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Sample records for aortic arch syndromes

  1. Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy

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    Elaheh Malakan Rad

    2014-01-01

    Full Text Available Goldenhar syndrome (GS or oculo-auriculo-vertebral dysplasia (OAVD, involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA, severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited.

  2. Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy

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    Elaheh Malakan Rad

    2014-01-01

    Goldenhar syndrome (GS) or oculo-auriculo-vertebral dysplasia (OAVD), involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA), severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and co...

  3. Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy.

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    Rad, Elaheh Malakan

    2014-09-01

    Goldenhar syndrome (GS) or oculo-auriculo-vertebral dysplasia (OAVD), involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA), severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited.

  4. Goldenhar syndrome with right circumflex aortic arch, severe coarctation and vascular ring in a twin pregnancy

    Science.gov (United States)

    Rad, Elaheh Malakan

    2014-01-01

    Goldenhar syndrome (GS) or oculo-auriculo-vertebral dysplasia (OAVD), involves a wide variety of organ systems. Cardiovascular anomalies are among the frequent malformations. The purpose of this report is to introduce a male case of a dizygotic twin pregnancy with GS and right circumflex aortic arch (RCAA), severe coarctation, hypoplastic aortic arch, aberrant right subclavian artery, vascular ring, bilateral renal artery stenosis, and mild Dandy-Walker syndrome. The embryology of RCAA and coarctation is revisited. PMID:25298700

  5. Common variable immunodeficiency syndrome with right aortic arch: a case report

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    Erbay Riza

    2004-02-01

    Full Text Available Abstract Background Common variable immunodificiency syndrome predominantly affects adults. It is characterized by low production of all the major classes of immunoglobulins. We report a case of common variable immunodeficiency syndrome with right aortic arch. An association of right-sided arch and common variable immunodificiency syndrome has not been previously reported. Case presentation A 41-year-old female patient presented with a history of recurrent pneumonia, sinusitis, otitis media, diarrhoea, cystitis since childhood. Biochemical and immunocytochemical analysis revealed common variable immunodeficiency syndrome and radiological evaluation confirmed right aortic arch and aberrant left subclavian artery. Conclusion Common variable immunodeficiency syndrome syndrome is a clinical entity that should be kept in mind in patients with recurrent infections of different sites.

  6. Double aortic arch with double aneuploidy-rare anomaly in combined Down and Klinefelter syndrome

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    Gerretsen, M.F.; Peelen, W.; Rammeloo, L.A.J.; Koolbergen, D.R.; Hruda, J.

    2009-01-01

    A 14-month-old boy with double aneuploidy and a double aortic arch suffered from frequently recurrent severe feeding and respiratory problems. Chromosomal analysis showed a 48,XXY + 21 karyotype: a double aneuploidy of Down syndrome (DS) and Klinefelter syndrome (KS). Only four cases of double aneup

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

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    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.

  8. Surgical management of a hypoplastic distal aortic arch and coarctation of aorta in a patient with Klippel-Feil syndrome, ascending aortic aneurysm and bicuspid aortic valve.

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    Sabol, Frantisek; Kolesar, Adrián; Toporcer, Tomás; Bajmoczi, Milan

    2014-10-01

    Klippel-Feil syndrome has been associated with cardiovascular malformations, but only 3 cases have been reported to be associated with aortic coarctation and surgical management is not defined. A 51-year old woman with Klippel-Feil syndrome associated with an aneurysm of the ascending aorta, hypoplastic aortic arch and aortic coarctation at the level of the left subclavian artery presented with shortness of breath 2 years after diagnosis. Imaging identified interim development of a 7.2-cm aneurysm at the level of the aortic coarctation. She underwent surgical repair with a Dacron interposition graft under hypothermic circulatory arrest. She continues to do well 18 months following repair.

  9. Central retinal artery occlusion following laser treatment for ocular ischemic aortic arch syndrome

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    Shah, Payal J.

    2015-12-01

    Full Text Available Objective: Ocular ischemic syndrome is a rare blinding condition generally caused by disease of the carotid artery. We describe a 69-year-old female with a 50 pack-year smoking history with aortic arch syndrome causing bilateral ocular ischemic syndrome. Methods: The patient presented with progressive visual loss and temple pain. Slit lamp biomicroscopy revealed bilateral iris neovascularization. This finding prompted a cardiovascular work up. Panretinal photocoagulation with retrobulbar block was performed in the right eye. Results: A temporal artery biopsy was negative. The carotid duplex sound showed only a 1–39% stenosis. MRA revealed a more proximal occlusion of the aortic branch for which she underwent subclavian carotid bypass surgery. At the one month follow up, the right eye suffered profound vision loss secondary to a central retinal artery occlusion. Conclusion: Ocular neovascularization may be one of the clinical manifestations of aortic arch syndrome. This case also illustrates the limitations of relying solely on carotid duplex ultrasound testing. We caution against overly aggressive panretinal photocoagulation utilizing retrobulbar anesthesia.

  10. Composite valve graft combined with replacement of the ascending aorta and aortic arch in a patient with Marfan's syndrome

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    黄方炯; 叶穗辉; 陈赤红; 杨禁非; 孙东; 吴强; 于建波

    2004-01-01

    @@ Modified Bentall procedure combined with total aortic arch replacement under deep hypothermic circulatory arrest (DHCA) and retrograde cerebral perfusion (RCP)is rarely reported. We performed this operation for a patient with worsened aortic regurgitation and cardiac shock. The operation involved the button method for coronary artery reconstruction, hypothermic circulatory arrest, and retrograde cerebral protection. The supraaortic branches were anastomosed to the prosthesis as an island flap.

  11. Mycotic Aneurysm of the Aortic Arch

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    Ji Hye Seo

    2014-08-01

    Full Text Available A mycotic aneurysm of the thoracic aorta is rare. We report a case of mycotic aneurysm that developed in the aortic arch. An 86-year-old man was admitted with fever and general weakness. Blood culture yielded methicillin-resistant Staphylococcus aureus. Chest X-ray showed an enlarged aortic arch, and computed tomography scan revealed an aneurysm in the aortic arch. The patient was treated only with antibiotics and not surgically. The size of the aneurysm increased rapidly, resulting in bronchial obstruction and superimposed pneumonia. The patient died of respiratory failure.

  12. Blood flow characteristics in the aortic arch

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    Prahl Wittberg, Lisa; van Wyk, Stevin; Mihaiescu, Mihai; Fuchs, Laszlo; Gutmark, Ephraim; Backeljauw, Philippe; Gutmark-Little, Iris

    2012-11-01

    The purpose with this study is to investigate the flow characteristics of blood in the aortic arch. Cardiovascular diseases are associated with specific locations in the arterial tree. Considering atherogenesis, it is claimed that the Wall Shear Stress (WSS) along with its temporal and spatial gradients play an important role in the development of the disease. The WSS is determined by the local flow characteristics, that in turn depends on the geometry as well as the rheological properties of blood. In this numerical work, the time dependent fluid flow during the entire cardiac cycle is fully resolved. The Quemada model is applied to account for the non-Newtonian properties of blood, an empirical model valid for different Red Blood Cell loading. Data obtained through Cardiac Magnetic Resonance Imaging have been used in order to reconstruct geometries of the the aortic arch. Here, three different geometries are studied out of which two display malformations that can be found in patients having the genetic disorder Turner's syndrome. The simulations show a highly complex flow with regions of secondary flow that is enhanced for the diseased aortas. The financial support from the Swedish Research Council (VR) and the Sweden-America Foundation is gratefully acknowledged.

  13. Complete interruption of the aortic arch

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    Park, Sung Hak; Park, In Kyu; Lee, Won Hwa; Kim, Yong Joo; Kang, Duk Sik; Lee, Jong Tae [Kyungpook National University College of Medicine, Taegu (Korea, Republic of)

    1984-09-15

    Complete interrupture of the aortic arch is one of the least common cardiac malformation. In this condition, continuity between the ascending and descending protions of the aorta is not present, and the descending aorta is supplied through a reversing patent ductus arteriosus. In the majority of the patients a ventricular septal defect is present. Authors have experienced a case of the complete interruption of aortic arch, interruption between the left carotid and the left subclavian artery.

  14. Supravalvular aortic stenosis in adult with anomalies of aortic arch vessels and aortic regurgitation

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    Valente, Acrisio Sales; Alencar, Polyanna; Santos, Alana Neiva; Lobo, Roberto Augusto de Mesquita; de Mesquita, Fernando Antônio; Guimarães, Aloyra Guedis

    2013-01-01

    The supravalvular aortic stenosis is a rare congenital heart defect being very uncommon in adults. We present a case of supravalvular aortic stenosis in adult associated with anomalies of the aortic arch vessels and aortic regurgitation, which was submitted to aortic valve replacement and arterioplasty of the ascending aorta with a good postoperative course. PMID:24598962

  15. Multislice CT angiography of interrupted aortic arch

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    Yang, Dong Hyun; Goo, Hyun Woo [Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea); Seo, Dong-Man; Yun, Tae-Jin; Park, Jeong-Jun [Asan Medical Center, University of Ulsan College of Medicine, Department of Paediatric Cardiac Surgery, Seoul (Korea); Park, In-Sook; Ko, Jae Kon; Kim, Young Hwee [Asan Medical Center, University of Ulsan College of Medicine, Department of Paediatric Cardiology, Seoul (Korea)

    2008-01-15

    Interrupted aortic arch (IAA) is defined as complete luminal and anatomic discontinuity between the ascending and descending aorta. Because almost all patients with IAA become critically ill during the neonatal period, they should undergo urgent corrective surgery. This clinical urgency necessitates a fast and accurate noninvasive diagnostic method. Although echocardiography remains the primary imaging tool for this purpose, it is not always sufficient for planning surgical correction of IAA, principally due to a limited acoustic window and the inexperience of imagers. In this context, multislice CT angiography is regarded as an appropriate imaging technique complementary to echocardiography because it is fast, accurate, and objective for the diagnosis of IAA. In this article we describe what cardiac radiologists should know about IAA in their clinical practice, including clinicopathological features, CT features with contemporary surgical methods and postoperative complications, and differentiation from coarctation of the aorta and aortic arch atresia. (orig.)

  16. Recurrent Pneumonia due to Double Aortic Arch

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    I. Sedighi

    2012-04-01

    Full Text Available Introduction: Pneumonia is one of the most common infections during childhood. In children with recurrent bacterial pneumonia complete evaluation for underlying factors is necessary. The most common underlying diseases include: antibody deficiencies , cystic fibrosis , tracheoesophageal fistula and increased pulmonary blood flow. Vascular ring and its pressure effect is a less common cause of stridor and recurrent pneumonia. Congenital abnormalities in aortic arch and main branches which form vascular ring around esophagus and trachea with variable pressure effect cause respiratory symptoms such as stridor , wheezing and recurrent pneumoniaCase Report: A 2 year old boy was admitted in our hospital with respiratory distress and cough . Chest x-Ray demonstrated right lobar pneumonia. He had history of stridor and wheezing from neonatal period and hospitalization due to pneumonia for four times. The patient received appropriate antibiotics. Despite fever and respiratory distress improvement, wheezing continued. Review of his medical documents showed fixed pressure effect on posterior aspect of esophagus in barium swallow. In CT angiography we confirmed double aortic arch.Conclusion: Double aortic arch is one of the causes of persistant respiratory symptom and recurrent pneumonia in children for which fluoroscopic barium swallow is the first non-invasive diagnostic method.(Sci J Hamadan Univ Med Sci 2012;19(1:70-74

  17. Early Diagnosis and Repair of Double Saccular Aneurysms of the Aortic Arch Associated With Aortic Coarctation in an Infant With Loeys-Dietz Syndrome.

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    Ilyin, Vladimir N; Kornoukhov, O Ju; Khovrin, Valery V; Kryukov, Vladislav A; Valitova, Asia A; Ilina, Maria V

    2016-03-01

    Multiple saccular aneurysms of the thoracic aorta in neonates and infants are exceedingly rare. An association of these aneurysms with Loeys-Dietz syndrome (LDS) in older age-groups is well known. This case report describes the diagnosis and subsequent successful repair of aortic coarctation associated with double saccular aneurysms of the thoracic aorta in patient with LDS during the first year of life.

  18. Aortic arch blunt injury in front-seat passenger.

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    Türkmen, Nursel; Eren, Bülent; Aydin, Şule Akköse

    2013-10-01

    Aortic arch blunt injury has highly lethal nature. Because the physical examination findings are subtle, immediate medical evaluation is very important. The case was a 72-year-old woman. Massive haemorrhage in the left haemotorax, contusion area in the left lung and a traumatic transection of the distal aortic arch was observed during autopsy. We described intersting autopsy case of aortic arch blunt injury.

  19. Right aortic arch with coarctation in Chinese children

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    Ming, Zhu; Aimin, Sun [Shanghai Children' s Medical Center, Department of Radiology, Shanghai (China)

    2008-05-15

    Because of the rarity of right aortic arch coarctation there are few reports of large groups of patients. To characterize the frequency and type of right aortic arch coarctation in a large group of pediatric patients. From June 1997 through May 2007, 11,276 consecutive children with congenital heart disease underwent multidetector CT (MDCT), MRI or angiocardiography examination. All children with a right aortic arch or coarctation were reviewed. Right aortic arch coarctation was found in 11 children representing 0.1% of the total group of 11,276 children, 1.7% of 658 children with native coarctations and 2.3% of 473 children with a right aortic arch. Among the 11 patients, 6 had long-segment narrowing and 7 had an aberrant left subclavian artery. MDCT, MRI and angiocardiography are reliable imaging techniques for the diagnosis of right aortic arch and coarctation. Our findings showed that the pattern of right aortic arch coarctation was different from that of left aortic arch coarctation, suggesting that they are different etiological entities. The pivotal role possibly played by flow dynamics in the development of right aortic arch coarctation is discussed. (orig.)

  20. Double aortic arch and nasogastric tubes: A fatal combination

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    Julia Massaad; Kelly Crawford

    2008-01-01

    Double aortic arch is a common form of complete vascular ring that encircles both the trachea and the esophagus, and presents with various respiratory and esophageal symptoms, usually in the pediatric population.We present a case of double aortic arch in an adult patient that manifested as massive upper gastrointestinal bleeding after prolonged nasogastric intubation.

  1. Adult presentation with vascular ring due to double aortic arch.

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    Kafka, Henryk; Uebing, Anselm; Mohiaddin, Raad

    2006-11-01

    This is a case report on the use of cardiovascular magnetic resonance imaging to diagnose vascular ring due to double aortic arch in an adult presenting with an abnormal chest X-ray. The experience in this case and the literature review identify the benefits of using cardiovascular magnetic resonance imaging to clarify complex aortic arch anatomy.

  2. A Fatal Aortoesophageal Fistula Caused by Critical Combination of Double Aortic Arch and Nasogastric Tube Insertion for Superior Mesenteric Artery Syndrome

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    Tomofumi Miura

    2010-06-01

    Full Text Available Double aortic arch (DAA is a rare vascular congenital abnormality. Since a vascular ring surrounds bronchus and esophagus, any oral or nasal intubation can physically cause fatal aortoesophageal fistula (AEF. We report herein the first case of association of DAA and superior mesenteric artery (SMA syndrome and the second case of AEF caused by nasogastric intubation in an adult with DAA. A 19-year-old woman visited our hospital for nausea and vomiting. She was diagnosed with SMA syndrome by computed tomography (CT. Nasogastric intubation relieved her symptoms in 4 days. Extramural compression with top ulceration was found in esophagogastroduodenoscopy on the 5th hospital day. She suddenly showed massive hematemesis on the 12th hospital day. AEF was found by CT. Soon, she died despite of intensive care. Retrospective interview disclosed the fact that DAA was pointed out in her childhood. We conclude that intubation must be avoided in DAA and a detailed clinical interview about DAA is mandatory to avoid AEF.

  3. Selective cerebro-myocardial perfusion in complex congenital aortic arch pathology: a novel technique.

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    De Rita, Fabrizio; Lucchese, Gianluca; Barozzi, Luca; Menon, Tiziano; Faggian, Giuseppe; Mazzucco, Alessandro; Luciani, Giovanni Battista

    2011-11-01

    Simultaneous cerebro-myocardial perfusion has been described in neonatal and infant arch surgery, suggesting a reduction in cardiac morbidity. Here reported is a novel technique for selective cerebral perfusion combined with controlled and independent myocardial perfusion during surgery for complex or recurrent aortic arch lesions. From April 2008 to April 2011, 10 patients with arch pathology underwent surgery (two hypoplastic left heart syndrome [HLHS], four recurrent arch obstruction, two aortic arch hypoplasia + ventricular septal defect [VSD], one single ventricle + transposition of the great arteries + arch hypoplasia, one interrupted aortic arch type B + VSD). Median age was 63 days (6 days-36 years) and median weight 4.0 kg (1.6-52). Via midline sternotomy, an arterial cannula (6 or 8 Fr for infants) was directly inserted into the innominate artery or through a polytetrafluoroethylene (PTFE) graft (for neonates cerebro-myocardial perfusion was 39 ± 18 min (17-69). Weaning from cardiopulmonary bypass was achieved without inotropic support in three and with low dose in seven patients. One patient required veno-arterial extracorporeal membrane oxygenation. Four patients, body weight cerebro-myocardial perfusion is feasible in patients with complex or recurrent aortic arch disease, starting from premature newborn less than 2.0 kg of body weight to adults. The technique is as safe as previously reported methods of cerebro-myocardial perfusion and possibly more versatile.

  4. A case of neonatal arterial thrombosis mimicking interrupted aortic arch.

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    Gürsu, Hazım Alper; Varan, Birgül; Oktay, Ayla; Özkan, Murat

    2015-06-01

    Neonatal arterial thrombosis is a very rare entity with clinical findings resembling coarctation of aorta or interrupted aortic arch. A two day-old male newborn was admitted to a different hospital with difficulty in sucking and sleepiness. On echocardiographic examination, a diagnosis of interrupted aortic arch was made and he was treated with prostoglandin E2. When the patient presented to our center, physical examination revealed that his feet were bilaterally cold. The pulses were not palpable and there were ecchymotic regions in the lower extremities. Echocardiography ruled out interrupted aortic arch. Computerized tomographic angiography revealed a large thrombosis and total occlusion of the abdominal aorta. Since there was no response to treatment with tissue plasminogen activator, we performed thrombectomy. Homozygous Factor V Leiden and Methylenetetrahydrofolate reductase mutations were found in this patient. Neonatal aortic thrombosis which is observed very rarely and fatal should be considered in the differential diagnosis of coarctation of aorta and interrupted aortic arch.

  5. Right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch

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    Ahn, Kyung-Sik; Yong, Hwan Seok; Woo, Ok Hee; Kang, Eun-Young [Korea University Guro Hospital, Department of Radiology, Seoul (Korea); Lee, Joo-Won [Korea University Guro Hospital, Department of Paediatrics, Seoul (Korea)

    2007-06-15

    We present a rare case of right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch. A 7-month-old boy presented with a cardiac murmur. Cardiac situs was normal and there was no evidence of an intracardiac shunt or patent ductus arteriosus. MR aortography revealed a right aortic arch that was high-positioned, tortuous and narrowed. This right aortic arch crossed the midline behind the oesophagus and continued as a left-sided descending aorta. The left common carotid and subclavian arteries arose from a large branching vascular structure that derived from the top of the left-sided descending aorta. The right common carotid artery arose from the ascending aorta. The proximal portion of the right common carotid artery showed very severe stenosis and poststenotic dilatation. The right subclavian artery originated distal to the narrowed and tortuous segment of the aortic arch. (orig.)

  6. Inflammatory aortic arch syndrome: contrast-enhanced, three-dimensional MR - angiography in stenotic lesions; Entzuendliches Aortenbogensyndrom: Stenosediagnostik mittels kontrastmittelverstaerkter 3D-MR-Angiographie im Vergleich mit der DSA

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    Both, M.; Mueller-Huelsbeck, S.; Biederer, J.; Heller, M.; Reuter, M. [Universitaetsklinikum Schleswig-Holstein, Kiel (Germany). Klinik fuer Diagnostische Radiologie; Reinhold-Keller, E.; Gross, W.L. [Rheumaklinik Bad Bramstedt GmbH (Germany)

    2004-01-01

    Purpose: To determine the value of contrast-enhanced, three-dimensional MR angiography for the evaluation of stenotic and occlusive vascular lesions in inflammatory aortic arch syndrome. Materials and Methods: 14 patients with inflammatory aortic arch syndrome (giant cell arteritis: n = 8, Takayasu arteritis: n = 4, ankylosing spondylitis: n = 1 sarcoidosis: n = 1) underwent MR angiography of the aortic arch and the supra-aortic vessels (n = 15,2 patients were examined twice) and of the abdominal aorta (n = 2). MRA was performed using a 3D-FLASH sequence (TR/TE 4.6/1.8 ms, flip angle 30 ) on a 1.5T system. MRA imaging was compared with the findings of DSA, which served as gold standard. Results: In a total of 467 examined vascular territories, DSA revealed 50 stenoses and 35 occlusions. All lesions were detected by MRA. In 23 segments, the degree of stenosis was overestimated by MRA. Sensitivity and specificity of MRA were 100% and 94,3%, positive and negative predictive values were 73.6 and 100%, and the accuracy was 95,1%. Conclusions: Despite a tendency to overestimate stenoses, contrast-enhanced three-dimensional MR angiography is a valid, non-invasive technique in the assessment of inflammatory aortic arch syndrome. (orig.) [German] Ziel: Bestimmung der Aussagekraft der kontrastmittelverstaerkten 3D-Magnetresonanzangiographie bei der Bewertung stenosierender und okkludierender Gefaessveraenderungen im Rahmen des entzuendlichen Aortenbogensyndroms. Material und Methoden: Bei 14 Patienten mit entzuendlichem Aortenbogensyndrom (Arteriitis temporalis: n = 8, Takayasu-Arteriitis: n = 4, Morbus Bechterew: n = 1, Sarkoidose: n = 1) erfolgte eine MRA des Aortenbogens und der supraaortalen Aeste (n = 15, darunter zwei Doppeluntersuchungen) sowie der Aorta abdominalis (n = 2). Zur Anwendung kam eine kontrastmittelverstaerkte 3D-FLASH-Sequenz (TR/TE 0,4/1,8 ms, Flipwinkel 30 ) an einem 1,5 Tesla-System. Die Befunde der MRA wurden mit der DSA als Referenzmethode

  7. A Review of Diseases of Aortic Arch: Diagnosis by CTA

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    S. Sabouri

    2007-05-01

    Full Text Available The noninvasive revolution in cardiovascular imaging has altered the diagnostic algorithm for all types of acquired and congenital cardiovascular disease. CT techniques are commonly used in the diagnosis of aortic arch and its major branch vessels as well as thoracic and abdominal aortic diseases. CT angiogra-phy combines with CT scans obtained detailed in-formation on precise morphology and extent of dis-ease. Studies were performed on an MDCT unit (4row GE light speed. In infants and small children, the seda-tion rate for CT was lower than that used for patients of similar age undergoing cardiac MR imaging. Seda-tion times ranged between 5 and 10 min, in the pedi-atric population, MDCT was performed with a 1- to 2.5-mm slice thickness in adults, MDCT was per-formed with a 2.5-mm slice thickness with 50% re-construction overlap, pitch 0.75-1.CT angiographic studies were performed with nonionic contrast mate-rial with iodine concentrations of 300 mg/mL admin-istered at a dose of 2-3 mL/kg. Contrast material was injected by power injection, followed by normal sa-line, an automated bolus-tracking technique was used at an injection rate of 2-4 mL/sec. the bolus-tracking device was placed on the ascending aorta. For pa-tients with thoracic outlet syndrome, CT angiogram was performed with neutral position of arm and ab-duction of the arm. Radiologist studied the CT image data in an axial cine paging mode as well as multiplanar reformations. To plan effective management of Aorta disease, CTA is displayed using a combination of 3D images, such as those obtained by multiplanar reformation (MPR, curved planar reformation (CPR, maximum intensity projection (MIP and volume rendering (VR. Case reports include coarctation of aorta, vascular ring, right sided aorta, interrupted aortic arch, abber-ant vessels, paitent ductus arteriosus, subclavian steel syndrome, aneurysm, dissection, thoracic outlet syn-drome, arthritis and atherosclerotic stenosis.

  8. Bare Metal Stenting for Endovascular Exclusion of Aortic Arch Thrombi

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    Mahnken, Andreas H., E-mail: mahnken@med.uni-marburg.de [University Hospital Giessen and Marburg, Philipps University of Marburg, Department of Diagnostic Radiology (Germany); Hoffman, Andras; Autschbach, Ruediger; Damberg, Anneke L. M., E-mail: anneke.damberg@rwth-aachen.de [University Hospital RWTH Aachen, Department of Thoracic, Cardiac and Vascular Surgery (Germany)

    2013-08-01

    BackgroundAortic thrombi in the ascending aorta or aortic arch are rare but are associated with a relevant risk of major stroke or distal embolization. Although stent grafting is commonly used as a treatment option in the descending aorta, only a few case reports discuss stenting of the aortic arch for the treatment of a thrombus. The use of bare metal stents in this setting has not yet been described.MethodsWe report two cases of ascending and aortic arch thrombus that were treated by covering the thrombus with an uncovered stent. Both procedures were performed under local anesthesia via a femoral approach. A femoral cutdown was used in one case, and a total percutaneous insertion was possible in the second case.ResultsBoth procedures were successfully performed without any periprocedural complications. Postoperative recovery was uneventful. In both cases, no late complications or recurrent embolization occurred at midterm follow-up, and control CT angiography at 1 respectively 10 months revealed no stent migration, freely perfused supra-aortic branches, and no thrombus recurrence.ConclusionTreating symptomatic thrombi in the ascending aorta or aortic arch with a bare metal stent is feasible. This technique could constitute a minimally invasive alternative to a surgical intervention or complex endovascular therapy with fenestrated or branched stent grafts.

  9. Management of hypoplastic aortic arch associated with neonatal coarctation.

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    Caspi, J; Ilbawi, M N; Muster, A; Roberson, D; Arcilla, R

    1994-12-01

    Controversy still exists as to whether hypoplastic aortic arch frequently associated with neonatal coarctation, should be enlarged at the time of coarctation repair. To determine the indications for and the outcome of repair of hypoplastic aortic arch, pre- and postoperative angiograms/echocardigraphy of 77 cases with isolated (n = 25, Group 1) or complex (n = 52, Group 2) neonatal coarctation operated upon between 1/80 and 12/89 were reviewed. Age was 5-14 days (mean 8 +/- 1.6). Aortic arch/ascending aorta diameter ratio (AR/AA) as a measure of the degree of aortic arch hypoplasia was 0.39-0.64 (0.52 +/- 0.04) in isolated and 0.15-0.47 (0.34 +/- 0.06) in complex coarctation (p < 0.05). Left subclavian flap aortoplasty was used in 72 patients; alone in 25, in combination with pulmonary artery banding in 43 patients, and simultaneously with intracardiac repair in 4 patients. Extensive reconstruction of aortic arch and coarctation with synthetic patch was performed in the remaining 5 patients (AR/AO = 0.16 +/- 0.03) using cardiopulmonary by-pass at the time of intracardiac repair. Operative mortality was 2/76 (2.5%). Follow-up is 6.6 +/- 1.4 years. Recoarctation occurred in 3 patients (4%). AR/AA increased to 0.86 +/- 0.1 in isolated (p < 0.05 vs preoperative) and to 0.7 +/- 0.1 in complex coarctation (p < 0.05 vs preoperative). In the majority of cases, hypoplastic aortic arch associated with coarctation grows satisfactorily following simple repair of coarctation with no significant residual narrowing.(ABSTRACT TRUNCATED AT 250 WORDS)

  10. Right-sided aortic arch with Kommerell′s aneurysm

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    Sanjay Orathi Patangi

    2014-01-01

    Full Text Available We present a case report of a 55-year-old lady who presented with progressive dysphagia and was diagnosed with a Kommerell′s aneurysm and a right-sided aortic arch. This case report outlines our management strategy and the challenges encountered during the perioperative period in a patient with this rare anomaly.

  11. Effect of aging on the configurational change of the aortic arch

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    Atsuhiro Kojima

    2016-04-01

    Full Text Available We explored the relationship between aging and the configuration of the aortic arch using computed tomography angiography (CTA. We retrospectively reviewed CTA obtained in 140 cases. The configuration of the aortic arch was categorized into three types based on the criteria mentioned by Madhwal et al., and the relationships between each configuration and patient characteristics were analyzed. Anomalies of the aortic arch were also explored. Twenty patients had a type-1 aortic arch (mean age, 56.1 years, 30 patients had a type-2 aortic arch (mean age, 66.3 years, and 89 patients had a type-3 aortic arch (mean age, 71.7 years. The mean age of patients with a type-3 aortic arch was significantly higher than that of patients with a type-1 aortic arch. No significant correlations between the type of aortic arch and other factors, such as smoking habit, were seen. The configuration of the aortic arch in our study appears to be significantly affected by the age of the patients.

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

    Science.gov (United States)

    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.

  13. Antenatal evaluation of fetal interrupted aortic arch type B

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    Ali Babacan

    2015-06-01

    Full Text Available Interruption of the aortic arch (IAA is a rare, severe form of congenital heart defect characterized by complete anatomical discontinuity between two adjacent segments of the aortic arch. The data on the features and outcomes of fetal IAA are limited. Three anatomical types have been described according to the site of interruption. The current recommendations for screening on the obstetric fetal anomaly scan include identification of a 4-chamber view, all 4 valves, and the outflow tracts, all of which can appear to be normal to the ultrasonographer in fetuses with conotruncal anomalies. Although the identification of IAA on a prenatal echocardiogram can be challenging, a number of anatomic features can facilitate the diagnosis. We aim to present the features and outcome of a case of IAA type B referred to our centre in the light of literatures.

  14. Congenital aortic arch anomalies: diagnosis using contrast enhanced magnetic resonance angiography

    Institute of Scientific and Technical Information of China (English)

    ZHU Ming; ZHONG Yu-min; LI Yu-hua; SUN Ai-min; JIN Biao

    2005-01-01

    @@ Congenital aortic arch anomalies occur most commonly in children. The disease can be classified into three types: ① obstructive congenital abnormalities, including coarctation of aorta (CoA) and interruption of aortic arch (IAA); ② non-obstructive congenital abnormalities, including double aortic arch and others; ③ congenital shunt abnormalities, including different types of patent ductus arteriosus (PDA). Management of patients with congenital aortic arch anomalies relies on imaging. Routine imaging modalities, such as conventional X-ray plain film and transthoracic echocardiography (TTE), have been recently complemented by magnetic resonance imaging (MRI).

  15. Contrast-enhanced magnetic resonance angiography of persistent fifth aortic arch in children

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    Zhong, Yumin; Zhu, Ming; Sun, Aimin; Li, Yuhua [Shanghai Jiao Tong University School of Medicine, Department of Radiology, Shanghai Children' s Medical Center, Shanghai (China); Jaffe, Richard B. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Gao, Wei [Shanghai Jiao Tong University School of Medicine, Department of Cardiology, Shanghai Children' s Medical Center, Shanghai (China)

    2007-03-15

    Cine angiography and echocardiography have been utilized to diagnose congenital aortic arch anomalies. However, the visualization of great vessels by echocardiography is limited, while cine angiography requires cardiac catheterization with ionizing radiation. Contrast-enhanced magnetic resonance angiography (MRA) is a noninvasive modality suitable for visualization of congenital aortic arch anomalies. To evaluate the utility of contrast-enhanced MRA in the diagnosis of persistent fifth aortic arch, a rare congenital aortic arch anomaly, and to compare the diagnostic accuracy of MRA with that of echocardiography and cine angiography. In four pediatric patients, contrast-enhanced MRA studies were performed for diagnosing persistent fifth aortic arch. The findings of MRA were compared with echocardiographic findings and confirmed by cine angiography and operation. Transthoracic surface echocardiography noted an aberrant vessel arising from the ascending aorta in two of four patients; the etiology of this vessel was uncertain. In the other two patients a diagnosis of coarctation was made. Of the four patients, only one was diagnosed with interruption of the aortic arch. Contrast-enhanced MRA clarified uncertain echocardiographic findings, enabling the correct diagnosis of persistent fifth aortic arch with fourth aortic arch interruption in all four patients. Contrast-enhanced MRA is a safe, accurate, and fast imaging technique for the evaluation of persistent fifth aortic arch and may obviate the need for conventional cine angiography. Cardiac catheterization may be reserved for some types of complicated congenital heart disease and for obtaining hemodynamic information. (orig.)

  16. [Relationship between aortic arch shape and blood pressure response after coarctation repair].

    Science.gov (United States)

    Ou, P; Mousseaux, E; Auriacombe, L; Pédroni, E; Balleux, F; Sidi, D; Bonnet, D

    2005-01-01

    The mechanisms of secondary hypertension after repair of coarctation of the aorta are not well understood. Abnormalities of the architecture of the aortic arch and their consequences on blood pressure have not been studied. In order to study the relationship between abnormalities or aortic arch architecture and resting blood pressure ninety-four patients without re-coarctation were followed up prospectively from 1997 to 2004 (mean age 16.9 +/- 8.1 years; mean weight 57.5 +/- 18.3 Kg; interval since surgery 16.3 +/- 5.4 years). All underwent MRI angiography of the thoracic aorta which enabled the abnormalities to be classified in 3 groups: gothic arch, crenellated arch and roman arch. Twenty-four patients (25.5%) were hypertensive and 70 (74.4%) normotensive. There were 40 gothic arches (42.5%). 14 crenellated arches (15%) and 40 roman arches (42.5%). Gothic arches were more commonly observed in the hypertensive patients (18/40, [45%, 95% CI 31-62]) than the crenellated arches (4/14, [28.5%, 95% CI 7-48]) or the roman arches (2/40, [5%, 95% CI 2-12]). Only the gothic arch was independently correlated with hypertension on multivariate analysis. The authors conclude that gothic deformation of the aortic arch is an independent predictive factor of hypertension in patients operated for coarctation with an excellent result on the isthmic region. Patients with a gothic appearance of their aortic arch should be followed up closely.

  17. A rare association of interrupted aortic arch type C and microdeletion 22q11.2.

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    Cuturilo, Goran; Drakulic, Danijela; Stevanovic, Milena; Jovanovic, Ida; Djukic, Milan; Miletic-Grkovic, Slobodanka; Atanaskovic-Markovic, Marina

    2008-10-01

    Microdeletion 22q11.2 is associated with a variety of findings, and the most common are cardiac defects. It is very frequently associated with interrupted aortic arch (IAA) type B and very rarely with type A and type C. Here we report the first case of IAA type C associated with 22q11.2 deletion in Serbia and, to the best of our knowledge, the fourth case described worldwide so far. By this report we would like to point out that all patients with IAA type C who have additional features specific for 22q11.2 microdeletion syndrome should be screened for the presence of this deletion.

  18. Axillary artery counter-current aortography in the newborn with aortic arch obstruction

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

    1989-08-01

    13 newborn infants with aortic coarctation were evaluated by counter-current aortographic technique. The right radial or brachial artery approach (2 cases in each group) did not give successful aortic arch imaging while the right axiallary artery approach resulted in adequate imaging in each of 9 cases investigated. In 2, the axillary artery was transiently weakened but returned to normal within 24 hours. No other complications were encountered. Axillary artery counter-current aortography is a safe and relatively non-invasive procedure which can be used to image the aortic arch in the newborn babies when other non-invasive diagnosis of aortic arch obstruction is tentative. (orig.).

  19. [Chronic type A aortic dissection associated with Turner syndrome; report of a case].

    Science.gov (United States)

    Tanaka, Hideyuki; Kozaki, Tomofumi; Kume, Masazumi; Miyamoto, Shinji

    2014-12-01

    Aortic dissection is a critical but rare complication of Turner syndrome. This report describes a case of chronic aortic dissection in a patient with Turner syndrome. A 54-year-old woman, suffering from mild back pain for 1 month, was referred to our hospital with a diagnosis of Stanford type A chronic aortic dissection and a bicuspid aortic valve with moderate regurgitation. Computed tomography revealed aortic dissection, involving all arch branches, extending from the ascending to the abdominal aorta. The true lumen of the brachial artery was nearly obstructed by the thrombosed false lumen. Elective aortic arch repair and aortic valve replacement were successfully performed. The patient was diagnosed with 45, XO Turner syndrome after surgery. Taking aortopathy of Turner syndrome into consideration, surveillance of the residual aorta was performed. No rapidly progressive dilatation of the residual aorta was detected during the 6 years' follow-up.

  20. [Interrupted aortic arch in a 68-year-old female with hypertension].

    Science.gov (United States)

    Benincasa, Susanna; Fineschi, Massimo; Ceccherini, Claudio; Pierli, Carlo

    2015-04-01

    Interrupted aortic arch (IAA) is a rare congenital malformation of the aorta and aortic arch. We report the case of a 68-year-old female with hypertension and poor control of blood pressure levels. She was diagnosed with aortic coarctation by aortography during young age. A double access angiography was performed that showed a type A IAA, a rare condition in adults that may cause hypertension.

  1. Unreliability of aortic size index to predict risk of aortic dissection in a patient with Turner syndrome

    Science.gov (United States)

    Nijs, Jan; Gelsomino, Sandro; Lucà, Fabiana; Parise, Orlando; Maessen, Jos G; Meir, Mark La

    2014-01-01

    Aortic size index (ASI) has been proposed as a reliable criterion to predict risk for aortic dissection in Turner syndrome with significant thresholds of 20-25 mm/m2. We report a case of aortic arch dissection in a patient with Turner syndrome who, from the ASI thresholds proposed, was deemed to be at low risk of aortic dissection or rupture and was not eligible for prophylactic surgery. This case report strongly supports careful monitoring and surgical evaluation even when the ASI is < 20 mm/m2 if other significant risk factors are present. PMID:24944765

  2. Right aortic arch with aberrant left innominate artery arising from Kommerell's diverticulum*

    Science.gov (United States)

    Faistauer, Ângela; Torres, Felipe Soares; Faccin, Carlo Sasso

    2016-01-01

    We report a case of an uncommon thoracic aorta anomaly-right aortic arch with aberrant left innominate artery arising from Kommerell's diverticulum-that went undiagnosed until adulthood. PMID:27777481

  3. Novel technique for arch and visceral artery debranching using ascending aortic inflow.

    Science.gov (United States)

    Mussa, Firas F; Walkes, Jon-Cecil; Lumsden, Alan B; Reardon, Michael J

    2008-01-01

    Aortic arch aneurysms extending proximally to the left subclavian artery have traditionally been approached through a median sternotomy and a staged elephant trunk procedure requiring a left thoracotomy or thoracoabdominal incision. We describe a novel technique for repair of such aneurysms in a single-stage, hybrid approach using bypass grafts arising from the ascending aorta to all arch and upper abdominal visceral arteries followed by endovascular exclusion of the aortic aneurysm.

  4. Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

    Science.gov (United States)

    Ergul, Yakup; Saygi, Murat; Ozyilmaz, Isa; Guzeltas, Alper; Odemis, Ender

    2015-01-01

    Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion. We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion. PMID:26175649

  5. Early Results of Chimney Technique for Type B Aortic Dissections Extending to the Aortic Arch

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    Huang, Chen [Affiliated Hospital of Nantong University, Department of General Surgery (China); Tang, Hanfei; Qiao, Tong; Liu, Changjian; Zhou, Min, E-mail: 813477618@qq.com [The Affiliated Hospital of Nanjing University Medical School, Department of Vascular Surgery, Nanjing Drum Tower Hospital (China)

    2016-01-15

    ObjectiveTo summarize our early experience gained from the chimney technique for type B aortic dissection (TBAD) extending to the aortic arch and to evaluate the aortic remodeling in the follow-up period.MethodsFrom September 2011 to July 2014, 27 consecutive TBAD patients without adequate proximal landing zones were retrograde analyzed. Chimney stent-grafts were deployed parallel to the main endografts to reserve flow to branch vessels while extending the landing zones. In the follow-up period, aortic remodeling was observed with computed tomography angiography.ResultsThe technical success rate was 100 %, and endografts were deployed in zone 0 (n = 3, 11.1 %), zone 1 (n = 18, 66.7 %), and zone 2 (n = 6, 22.2 %). Immediately, proximal endoleaks were detected in 5 patients (18.5 %). During a mean follow-up period of 17.6 months, computed tomography angiography showed all the aortic stent-grafts and chimney grafts to be patent. Favorable remodeling was observed at the level of maximum descending aorta and left subclavian artery with expansion of true lumen (from 18.4 ± 4.8 to 25 ± 0.86 mm, p < 0.001 and 27.1 ± 0.62 to 28.5 ± 0.37 mm, p < 0.001) and depressurization of false lumen (from 23.7 ± 2.7 to 8.7 ± 3.8 mm, p < 0.001, from 5.3 ± 1.2 to 2.1 ± 2.1 mm, p < 0.001). While at the level of maximum abdominal aorta, suboptimal remodeling of the total aorta (from 24.1 ± 0.4 to 23.6 ± 1.5 mm, p = 0.06) and true lumen (from 13.8 ± 0.6 to 14.5 ± 0.4 mm, p = 0.08) was observed.ConclusionBased on our limited experience, the chimney technique with thoracic endovascular repair is demonstrated to be promising for TBAD extending to the arch with favorable aortic remodeling.

  6. Development of the human aortic arch system captured in an interactive three-dimensional reference model.

    Science.gov (United States)

    Rana, M Sameer; Sizarov, Aleksander; Christoffels, Vincent M; Moorman, Antoon F M

    2014-06-01

    Variations and mutations in the human genome, such as 22q11.2 microdeletion, can increase the risk for congenital defects, including aortic arch malformations. Animal models are increasingly expanding our molecular and genetic insights into aortic arch development. However, in order to justify animal-to-human extrapolations, a human morphological, and molecular reference model would be of great value, but is currently lacking. Here, we present interactive three-dimensional reconstructions of the developing human aortic arch system, supplemented with the protein distribution of developmental markers for patterning and growth, including T-box transcription factor TBX1, a major candidate for the phenotypes found in patients with the 22q11.2 microdeletion. These reconstructions and expression data facilitate unbiased interpretations, and reveal previously unappreciated aspects of human aortic arch development. Based on our reconstructions and on reported congenital anomalies of the pulmonary trunk and tributaries, we postulate that the pulmonary arteries originate from the aortic sac, rather than from the sixth pharyngeal arch arteries. Similar to mouse, TBX1 is expressed in pharyngeal mesenchyme and epithelia. The endothelium of the pharyngeal arch arteries is largely negative for TBX1 and family member TBX2 but expresses neural crest marker AP2α, which gradually decreases with ongoing development of vascular smooth muscle. At early stages, the pharyngeal arch arteries, aortic sac, and the dorsal aortae in particular were largely negative for proliferation marker Ki67, potentially an important parameter during aortic arch system remodeling. Together, our data support current animal-to-human extrapolations and future genetic and molecular analyses using animal models of congenital heart disease. © 2013 Wiley Periodicals, Inc.

  7. Aortic arch thrombectomy in a 2.8 kilogram neonate--a case report and review of the literature.

    Science.gov (United States)

    Omeje, Ikenna; Ram, Awat; Kostolny, Martin

    2013-02-01

    Aortic arch thrombus is a rare occurrence in neonates. In the few described cases, this has mainly been associated with sepsis or early postnatal interventions, such as insertion of umbilical arterial line. We describe a case of occlusive aortic arch thrombus in a neonate who presented with signs of critical coarctation and successfully underwent surgical thrombectomy on deep hypothermic circulatory arrest. We also present a review of the most recently published cases of aortic arch thrombus in neonates and the treatment options employed.

  8. The origin and significance of secondary flows in the aortic arch.

    Science.gov (United States)

    Black, M M; Hose, D R; Lawford, P V

    1995-01-01

    This paper comprises a study of the secondary flow patterns that can develop in the human aortic arch. Clinical evidence of these secondary flows has been obtained by Kilner et al. using magnetic resonance velocity mapping techniques. Some of their results are presented for comparison in this paper. Four difference parametric models of the aortic arch have been analysed using computational fluid dynamic techniques. Both steady and transient flow conditions have been considered and two different commercially available software packages were used, namely FIDAP and FLOTRAN. A satisfactory comparison of the theoretical analysis with the results, both in vivo and in vitro, obtained by Kilner et al. for their out-of-plane inlet model was found. The theoretical analysis can now be extended to analyse the effect of different configurations and orientations of artificial aortic valves on the resulting aortic arch flow patterns.

  9. 57. Growth of left ventricular outflow tract after repair of ventricular septal defect and aortic arch obstruction

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    Abdulraouf Jijeh

    2015-10-01

    Conclusion: There is significant LVOT growth expected after repair of VSD and aortic arch obstruction. Small aortic valve and LVOT at diagnosis of those cases are not correlating with the need of surgical reintervention for LVOT obstruction.

  10. Role of aortic arch vascular mechanics in cardiovagal baroreflex sensitivity.

    Science.gov (United States)

    Klassen, Stephen A; Chirico, Daniele; Dempster, Kylie S; Shoemaker, J Kevin; O'Leary, Deborah D

    2016-07-01

    Cardiovagal baroreflex sensitivity (cvBRS) measures the efficiency of the cardiovagal baroreflex to modulate heart rate in response to increases or decreases in systolic blood pressure (SBP). Given that baroreceptors are located in the walls of the carotid sinuses (CS) and aortic arch (AA), the arterial mechanics of these sites are important contributors to cvBRS. However, the relative contribution of CS and AA mechanics to cvBRS remains unclear. This study employed sex differences as a model to test the hypothesis that differences in cvBRS between groups would be explained by the vascular mechanics of the AA but not the CS. Thirty-six young, healthy, normotensive individuals (18 females; 24 ± 2 yr) were recruited. cvBRS was measured using transfer function analysis of the low-frequency region (0.04-0.15 Hz). Ultrasonography was performed at the CS and AA to obtain arterial diameters for the measurement of distensibility. Local pulse pressure (PP) was taken at the CS using a hand-held tonometer, whereas AA PP was estimated using a transfer function of brachial PP. Both cvBRS (25 ± 11 vs. 19 ± 7 ms/mmHg, P = 0.04) and AA distensibility (16.5 ± 6.0 vs. 10.5 ± 3.8 mmHg(-1) × 10(-3), P = 0.02) were greater in females than males. Sex differences in cvBRS were eliminated after controlling for AA distensibility (P = 0.19). There were no sex differences in CS distensibility (5.32 ± 2.3 vs. 4.63 ± 1.3 mmHg(-1) × 10(-3), P = 0.32). The present data demonstrate that AA mechanics are an important contributor to differences in cvBRS.

  11. Critical thrombotic obstruction of transverse aortic arch in a neonate

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    Karunakar Vadlamudi

    2015-01-01

    Full Text Available A 3-day-old neonate presented with features suggestive of coarctation of aorta. Echocardiography showed a large organized thrombus in the transverse arch causing obstruction to theaortic arch and carotids with partial recanalization. Patient underwent surgical thromboendarterectomy with arch reconstruction. The evaluation did not reveal any hematological abnormalities and suspected to be due to fetal thromboembolism. Patient improved well, and no neurological deficits were observed during follow-up.

  12. Right aortic arch with aberrant left innominate artery: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Midiri, M. [Istituto di Radiologia, Policlinico Universitario, Bari (Italy); Finazzo, M.; Lagalla, R.; De Maria, M. [Istituto di Radiologia ``Pietro Cignolini``, Policlinico Universitario, Palermo (Italy); Pilato, M. [Reparto di Cardiochirurgia, Palermo (Italy)

    1999-03-01

    A rare case of a 60-year-old man with a right aortic arch and aberrant left innominate artery is presented. This case had an unusual clinical presentation. The dysphagia appeared suddenly in adulthood, whereas vascular rings, when symptomatic, usually manifest early in childhood.To our knowledge, MR imaging findings of this anomaly have never been reported. The diagnosis was made by MR imaging and confirmed by surgery. Magnetic resonance imaging can replace angiography in the assessment of the aortic arch anomalies. (orig.) With 4 figs., 11 refs.

  13. Cardiogenic shock due to coronary artery disease associated with interrupted aortic arch

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    Luís Alberto Oliveira Dallan

    2013-06-01

    Full Text Available Acute pulmonary edema is a serious event. Its occurrence in association with interrupted aortic arch and coronary heart disease is rare. Recently, an old patient developed cardiogenic shock and acute pulmonary edema due to acute coronary insufficiency, associated with interrupted aortic arch. The coronary angiography revealed occlusion of the right coronary artery and 95% obstruction in the left main coronary artery, associated with interruption of the descending aorta. Coronary artery bypass graft was performed, without extracorporeal circulation, to the anterior descending coronary artery. We discuss the initial management, given the seriousness of the case.

  14. Invasive aspergillosis in the aortic arch with infectious Aspergillus lesions in pulmonary bullae

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    Isao Watanabe

    2015-03-01

    Full Text Available A patient with pulmonary bullae died of massive hemoptysis. At autopsy a hole was observed in the aortic wall. A microscopic examination indicated small Aspergillus lesions in pulmonary bullae and extensive necrotic lesions with Aspergillus hyphae in the media of the thoracic aorta. These findings led to a diagnosis of invasive aspergillosis in the aortic arch. This is a rare case in which Aspergillus invaded the aorta in a patient without hematologic neoplasms or neutropenia.

  15. [Application of multiple-branch prostheses in reconstruction of the aortic arch in DeBakey Type I aortic dissection].

    Science.gov (United States)

    Cherniavskiĭ, A M; Alsov, S A; Marchenko, A V; Smolianinov, K A; Sizov, G G; Zotov, A S

    2006-01-01

    Over the period from January 2002 to March 2005, a total of 78 patients were operated on for DeBakey type I aortic dissection. Of these, five patients underwent prosthetic reconstruction of the aortic arch and brachiocephalic arteries (BCA), performed with the help of the Dacron prosthesis Gelweave four-branch plexus Vascutek. The major indication for using this prosthesis was the extending of the dissection into the BC As, with various degree of obstruction thereof. The overwhelming majority of the patients were men (80 %). The patients' average age amounted to 44.3+/-5.6 years (ranging from 36 to 57 years). In three cases, the aortic dissection was of chronic course, with acute and subacute dissection being diagnosed in the remaining two cases. The median-sternotomy access, and additional approaches to the left and right common carotid arteries were used in order to carry out prosthetic reconstruction of the ascending branch, aortic arch, right subclavian artery, right common carotid artery (CCA) and left CCA with the help of the multiple-branch prosthesis. Protection of the brain consisted of craniocerebral hypothermia, hypothermal circulatory arrest in a combination with retrograde cerebral perfusions through the vena cava superior. The duration of the circulatory arrest averagely amounted to 59.2+/-4 min, with the mean time of artificial circulation equalling 230.6+/-19.7 min, and the time of aortic occlusion was 193.1+/-11 minutes. None of the patients developed either neurological or haemorrhagic complications postoperatively. Hence, the surgical technique using multiple-branch prostheses for prosthetic reconstruction of the aortic arch in type I aortic dissection with BCA obstruction proved to be effective and safe.

  16. Total aortic arch replacement: superior ventriculo-arterial coupling with decellularized allografts compared with conventional prostheses.

    Directory of Open Access Journals (Sweden)

    Alexander Weymann

    Full Text Available To date, no experimental or clinical study provides detailed analysis of vascular impedance changes after total aortic arch replacement. This study investigated ventriculoarterial coupling and vascular impedance after replacement of the aortic arch with conventional prostheses vs. decellularized allografts.After preparing decellularized aortic arch allografts, their mechanical, histological and biochemical properties were evaluated and compared to native aortic arches and conventional prostheses in vitro. In open-chest dogs, total aortic arch replacement was performed with conventional prostheses and compared to decellularized allografts (n = 5/group. Aortic flow and pressure were recorded continuously, left ventricular pressure-volume relations were measured by using a pressure-conductance catheter. From the hemodynamic variables end-systolic elastance (Ees, arterial elastance (Ea and ventriculoarterial coupling were calculated. Characteristic impedance (Z was assessed by Fourier analysis.While Ees did not differ between the groups and over time (4.1±1.19 vs. 4.58±1.39 mmHg/mL and 3.21±0.97 vs. 3.96±1.16 mmHg/mL, Ea showed a higher increase in the prosthesis group (4.01±0.67 vs. 6.18±0.20 mmHg/mL, P<0.05 in comparison to decellularized allografts (5.03±0.35 vs. 5.99±1.09 mmHg/mL. This led to impaired ventriculoarterial coupling in the prosthesis group, while it remained unchanged in the allograft group (62.5±50.9 vs. 3.9±23.4%. Z showed a strong increasing tendency in the prosthesis group and it was markedly higher after replacement when compared to decellularized allografts (44.6±8.3 dyn·sec·cm(-5 vs. 32.4±2.0 dyn·sec·cm(-5, P<0.05.Total aortic arch replacement leads to contractility-afterload mismatch by means of increased impedance and invert ventriculoarterial coupling ratio after implantation of conventional prostheses. Implantation of decellularized allografts preserves vascular impedance thereby improving

  17. Right-sided aortic arch with anomalous origin of the left subclavian artery: Case report

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    Vučurević Goran

    2011-01-01

    Full Text Available Introduction. A right-sided aortic arch is a rare congenital defect of the aorta with incidence of 0.05% to 0.1% reported in published series. Usually it is associated with congenital heart anomalies and esophageal and tracheal compression symptoms. We present a case of a right-sided aortic arch of anomalous left subclavian artery origin, accidentally revealed during multislice CT (MSCT supraaortic branches angiography. Case Outline. A 53-year-old female patient was examined at the Outpatients’ Unit of the Vascular Surgery University Clinic for vertigo, occasional dizziness and difficulty with swallowing. Physical examination revealed a murmur of the left supraclavicular space, with 15 mmHg lower rate of arterial tension on the left arm. Ultrasound of carotid arteries revealed 60% stenosis of the left subclavian artery and bilateral internal carotid artery elongation. MSCT angiography revealed a right-sided aortic arch with aberrant separation of the left subclavian artery that was narrowed 50%, while internal carotid arteries were marginally elongated. There was no need for surgical treatment or percutaneous interventions, so that conservative treatment was indicated. Conclusion. A right-sided aortic arch is a very rare anomaly of the location and branching of the aorta. Multislice CT angiography is of great importance in the diagnostics of this rare disease.

  18. Stenting of Variant Left Carotid Artery Using Brachial Artery Approach in a Patient with Unusual Type of Bovine Aortic Arch

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    Emre Gürel

    2016-01-01

    Full Text Available Bovine aortic arch is the most frequently encountered variation in human aortic arch branching. A 63-year-old Asian male presented with symptomatic severe stenosis of left carotid artery originating from the brachiocephalic trunk. Selective engagement to the left carotid artery was unsuccessful using transfemoral approach. We reported on a successful left carotid artery stenting case using right brachial artery approach in a bovine aortic arch. This paper is worthy of reporting in terms of guiding physicians for interventional procedures in these types of challenging cases.

  19. Interrupted aortic arch diagnosed by ECG-gated multi-slice computed tomography angiography: a case report

    Institute of Scientific and Technical Information of China (English)

    ZHOU Yang-yang; HAN Ping; FENG Gan-sheng; LIANG Bo

    2005-01-01

    @@ Interrupted aortic arch (IAA) is a rare congenital cardiovascular disease with major intracardiac defects and always with multisystem non-cardiac malformations. It occurs in 1: 10,000 births, and about 1% of the patients with congenital heart defects.

  20. Video-assisted thoracoscopic left lower lobectomy in a patient with lung cancer and a right aortic arch

    Directory of Open Access Journals (Sweden)

    Wada Hideyuki

    2012-11-01

    Full Text Available Abstract A right aortic arch is a rare congenital anomaly, with a reported incidence of around 0.1%. A patient with a right aortic arch underwent video-assisted thoracic surgery left lower lobectomy and mediastinal lymph node dissection for squamous cell carcinoma. There was no aortic arch or descending aorta in the left thoracic cavity, but the esophagus. There was no anomaly in the location or branching of the pulmonary vessels, the bronchi, and the lobulation of the lungs. The vagus nerve was found at the level of the left pulmonary artery. The arterial ligament was found between the left subclavian artery and the left pulmonary artery. The recurrent laryngeal nerve was recurrent around the left subclavian artery. A Kommerell diverticulum was found at the origin of the left subclavian artery. The patient experienced no complications. We conclude that video-assisted thoracoscopic lobectomy with mediastinal dissection is feasible for treating lung cancer with a right aortic arch.

  1. Pattern-based approach to fetal congenital cardiovascular anomalies using the transverse aortic arch view on prenatal cardiac MRI

    Energy Technology Data Exchange (ETDEWEB)

    Dong, Su-Zhen; Zhu, Ming [Shanghai Jiaotong University School of Medicine, Department of Radiology, Shanghai Children' s Medical Center, Shanghai (China)

    2015-05-01

    Fetal echocardiography is the imaging modality of choice for prenatal diagnosis of congenital cardiovascular anomalies. However, echocardiography has limitations. Fetal cardiac magnetic resonance imaging (MRI) has the potential to complement US in detecting congenital cardiovascular anomalies. This article draws on our experience; it describes the transverse aortic arch view on fetal cardiac MRI and important clues on an abnormal transverse view at the level of the aortic arch to the diagnosis of fetal congenital cardiovascular anomalies. (orig.)

  2. Aortic Arch Atherosclerosis——An Important Potential Source Of Cerebral Embolic Stroke

    Institute of Scientific and Technical Information of China (English)

    Yi Guo; Shao wen Zhang; Si Chen

    2000-01-01

    Background and purpose: Recent years with use of transesophageal echography renew awareness of aortic atherosclerosis. The purpose of this study was to determine the prevalence, clinical significance, and embolic potential of thoracic aortic plaque in patients with cerebral emblism; and further study the correlation aortic plaque with carotid or heart disease. Method: 49 consecutive patients with cerebral embolism was included in this sutdy. We uscd TEE to evaluated potential source of emboli in aortic arch and heart, and duplex in carotid artery. A atherosclerotic lesion of thoracic aorta was defined as normal(O); mild plaque (1); moderate plaque (2); protruding plaque or mobile plaque (3). Result: 31(63%) patients showed evidence of AAA; 7 (14.3%) patients had mild AAA, 9 (18.4%) patients had moderate AAA and 15 (32.7%) patients had severe AAA, In these 15 patients 11 patients neither severe ICAA or embolic heart disease, the AAA may be responsible to the cerebral embolism; 33 patients had internal carotid arterial atherosclerosis(ICAA), 9 patients had embolic heart disease; Age, ICAA had significant correlated with aortic plaque. Conclusion: Aortic atherosclerosis is common in cerebral embolism. Aortic plaque might be not only responsible for some unexplained embolic event, but also for some of the embolic stroke in the group of patient do have carotid artery or heart disease. Age might be important risk factor for the development of atherosclerotic lesion in the thoracic aorta.

  3. Magnetic resonance imaging of coarctation of the aorta and postoperative interrupted aortic arch

    Energy Technology Data Exchange (ETDEWEB)

    Kakizawa, Hideyuki; Tanaka, Takashi; Takada, Osamu; Nakayama, Shingo; Ogata, Hiroshi; Zuguchi, Masayuki (Tohoku Univ., Sendai (Japan). School of Medicine)

    1991-03-01

    ECG-gated MRI was performed at 1.5 T on 9 patients with coarctation of the aorta and restensis of the aorta after previous aortoplasty for coarctation of the aorta or interrupted aortic arch. The age of the patients ranged from 7 days to 3.3 years. MRI was more useful in assessing the severity of stenosis than echocardiography. Four patients had balloon dilation angioplasty for restenosis of the aorta. MRI was also useful in deciding the appropriate balloon size for angioplasty, and follow up after treatment. However, MRI could not always visualize the whole lesion in one slice, especially when the course of the aortic arch was not on the same plane. (author).

  4. Left-Sided Patent Ductus Arteriosus in a Right-Sided Aortic Arch

    Directory of Open Access Journals (Sweden)

    Ming-Yen Ng

    2014-01-01

    Full Text Available We present a 31-year-old female with repaired tetralogy of Fallot (TOF and right-sided aortic arch (RAA with left-sided patent ductus arteriosus (PDA originating from the left brachiocephalic artery. This is a rare finding but most common site for a PDA in TOF and a RAA. To the best of our knowledge, this is the first demonstration of this rare finding on MRI in the literature.

  5. Right-Sided Aortic Arch with Aberrant Left Subclavian Artery from Kommerell's Diverticulum

    Directory of Open Access Journals (Sweden)

    M.Y. Mubarak

    2011-06-01

    Full Text Available A previously healthy 52-year-old man had a chest radiograph for medical check-up and found to have a right-sided aortic arch. Computed tomography of the thorax revealed a right-sided aorticarch with aberrant left subclavian artery originated from Kommerell's diverticulum. Barium swallow examination showed compression of the posterior wall of the esophagus. He was asymptomatic and no surgical intervention was performed.

  6. Hybrid palliation of interrupted aortic arch in a high-risk neonate

    Directory of Open Access Journals (Sweden)

    Karimi Mohsen

    2010-01-01

    Full Text Available We report a case of a high-risk neonate with interrupted aortic arch (IAA and ventricular septal defect who underwent a successful hybrid palliative procedure using a ductal stent and bilateral branch pulmonary artery banding. This case represents not only a successful use of hybrid approach in high-risk neonates with IAA, but also introduces an alternative and safe access for ductal stent insertion through the right ventricular infundibulum.

  7. Successful Anticoagulation Therapy for Antiphospholipid Syndrome with Mobile Aortic Thrombi

    Science.gov (United States)

    Park, Hyun Oh; Moon, Seong Ho; Kim, Jong Woo; Byun, Joung Hun; Kim, Sung Hwan; Yang, Jun Ho; Lee, Chung-Eun; Kim, Jong-Duk

    2016-01-01

    Hypercoagulable states have been associated with aortic thrombosis. Antiphospholipid syndrome (APS) is one of the commonest types of acquired thrombophilia. We report the case of successful anticoagulation management in an APS patient with mobile thrombi within the aorta. A 58-year-old male patient presented to the emergency department (ED) with right-sided hemiparesis. His first symptoms were noted approximately 12–16 hours before presentation to the ED. Magnetic resonance imaging of the brain showed acute embolic infarction of the left frontal and parietotemporal lobes. Transesophageal echocardiography (TEE) and computed tomography angiography (CTA) demonstrated mobile thrombi attached to the wall of the ascending aorta and aortic arch. The patient was diagnosed with APS based on positivity of anti-beta-2 glycoprotein 1 antibodies, and was initiated on anticoagulation therapy. Repeated TEE and CTA revealed complete resolution of the thrombi after 12 days of treatment; the patient was discharged well. PMID:28042559

  8. Retrograde cerebral perfusion (RCP) in aortic arch surgery: efficacy and possible mechanisms of brain protection.

    Science.gov (United States)

    Bavaria, J E; Pochettino, A

    1997-07-01

    Retrograde cerebral perfusion (RCP) was first introduced to treat air embolism during cardiopulmonary bypass (CPB). Its use was reintroduced to extend the safety of hypothermic circulatory arrest (HCA) during operations involving an open aortic arch. RCP seems to prevent cerebral rewarming during HCA. Both clinical and animal data suggest that RCP provides between 10% and 30% of baseline cerebral blood flow when administered through the superior vena cava (SVC) at jugular pressures of 20 to 25 mm Hg. RCP flows producing jugular venous pressures higher than 30 mm Hg may cause cerebral edema. Cerebral blood flow generated by RCP is able to sustain some cerebral metabolic activity, yet is not able to fully meet cerebral energy demands even at temperatures of 12 degrees to 18 degrees C. RCP may further prevent embolic events during aortic arch surgery when administered at moderate jugular vein pressures (RCP, when applied during aortic arch reconstruction, may extend the safe HCA period and improve morbidity and mortality, especially when HCA times are more than 60 minutes. RCP applied in patients and severe carotid and brachiocephalic occlusive disease may be ineffective, and caution is in order when RCP times are greater than 90 minutes.

  9. Multimodal optical measurement in vitro of surface deformations and wall thickness of the pressurized aortic arch.

    Science.gov (United States)

    Genovese, Katia; Humphrey, Jay D

    2015-04-01

    Computational modeling of arterial mechanics continues to progress, even to the point of allowing the study of complex regions such as the aortic arch. Nevertheless, most prior studies assign homogeneous and isotropic material properties and constant wall thickness even when implementing patient-specific luminal geometries obtained from medical imaging. These assumptions are not due to computational limitations, but rather to the lack of spatially dense sets of experimental data that describe regional variations in mechanical properties and wall thickness in such complex arterial regions. In this work, we addressed technical challenges associated with in vitro measurement of overall geometry, full-field surface deformations, and regional wall thickness of the porcine aortic arch in its native anatomical configuration. Specifically, we combined two digital image correlation-based approaches, standard and panoramic, to track surface geometry and finite deformations during pressurization, with a 360-deg fringe projection system to contour the outer and inner geometry. The latter provided, for the first time, information on heterogeneous distributions of wall thickness of the arch and associated branches in the unloaded state. Results showed that mechanical responses vary significantly with orientation and location (e.g., less extensible in the circumferential direction and with increasing distance from the heart) and that the arch exhibits a nearly linear increase in pressure-induced strain up to 40%, consistent with other findings on proximal porcine aortas. Thickness measurements revealed strong regional differences, thus emphasizing the need to include nonuniform thicknesses in theoretical and computational studies of complex arterial geometries.

  10. Biomechanical implications of excessive endograft protrusion into the aortic arch after thoracic endovascular repair.

    Science.gov (United States)

    Rinaudo, Antonino; Raffa, Giuseppe Maria; Scardulla, Francesco; Pilato, Michele; Scardulla, Cesare; Pasta, Salvatore

    2015-11-01

    Endografts placed in the aorta for thoracic endovascular aortic repair (TEVAR) may determine malappositioning to the lesser curvature of the aortic wall, thus resulting in a devastating complication known as endograft collapse. This premature device failure commonly occurs in young individuals after TEVAR for traumatic aortic injuries as a result of applications outside the physical conditions for which the endograft was designed. In this study, an experimentally-calibrated fluid-structure interaction (FSI) model was developed to assess the hemodynamic and stress/strain distributions acting on the excessive protrusion extension (PE) of endografts deployed in four young patients underwent TEVAR. Endograft infolding was experimentally measured for different hemodynamic scenarios by perfusion testing and then used to numerically calibrate the mechanical behavior of endograft PE. Results evinced that the extent of endograft can severely alter the hemodynamic and structural loads exerted on the endograft PE. Specifically, PE determined a physiological aortic coarctation into the aortic arch characterized by a helical flow in the distal descending aorta. High device displacement and transmural pressure across the stent-graft wall were found for a PE longer than 21 mm. Finally, marked intramural stress and principal strain distributions on the protruded segment of the endograft wall may suggest failure due to material fatigue. These critical parameters may contribute to the endograft collapse observed clinically and can be used to design new devices more suitable for young individuals to be treated with an endoprosthesis for TEVAR of blunt traumatic aortic injuries.

  11. Carotid stenting through the right brachial approach for left internal carotid artery stenosis and bovine aortic arch configuration

    Energy Technology Data Exchange (ETDEWEB)

    Montorsi, Piero; Galli, Stefano; Ravagnani, Paolo; Ghulam Ali, Sarah; Trabattoni, Daniela; Fabbiocchi, Franco; Lualdi, Alessandro; Ballerini, Giovanni; Andreini, Daniele; Pontone, Gianluca; Annoni, Andrea; Bartorelli, Antonio L. [Institute of Cardiology University of Milan, Centro Cardiologico Monzino, IRCCS, Milan (Italy)

    2009-08-15

    Unfavorable complex anatomy or congenital anomalies of supra-aortic vessel take-off may increase carotid artery stenting (CAS) procedural difficulties and complications through the femoral route. We assessed the feasibility, safety, and efficacy of CAS through the right brachial approach in patients in whom left internal carotid artery stenosis and bovine aortic arch configuration were identified with computed tomography (CT) angiography. Bovine configuration of the aortic arch and left carotid artery stenosis were easily identified by CT angiography and successfully treated through the right brachial approach technique. (orig.)

  12. Aortic events in a nationwide Marfan syndrome cohort

    DEFF Research Database (Denmark)

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

    2016-01-01

    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...

  13. Aortic root and proximal aortic arch replacement (performed by a left-handed surgeon).

    Science.gov (United States)

    Carrel, Thierry

    2017-01-01

    We present our standard technique of composite graft replacement performed by a left-handed surgeon. This procedure is performed with a 30-day mortality comparable to that of elective isolated aortic valve replacement.

  14. Importance of stent-graft design for aortic arch aneurysm repair

    Directory of Open Access Journals (Sweden)

    C Singh

    2017-02-01

    Full Text Available Aneurysm of the aorta is currently treated by open surgical repair or endovascular repair. However, when the aneurysm occurs in regions between the aortic arch and proximal descending aorta, it can be a complex pathology to treat due to its intricate geometry. When complex aortic aneurysms are treated with the conventional procedures, some of the patients present with significant post-operative complications and high mortality rate. Consequently, a clinically driven hybrid innovation known as the frozen elephant trunk procedure was introduced to treat complex aortic aneurysms. Although this procedure significantly reduces mortality rate and operating time, it is still associated with complications such as endoleaks, spinal cord ischemia, renal failure and stroke. Some of these complications are consequences of a mismatch in the biomechanical behaviour of the stent-graft device and the aorta. Research on complex aneurysm repair tended to focus more on the surgical procedure than the stent-graft design. Current stent-graft devices are suitable for straight vessels. However, when used to treat aortic aneurysm with complex geometry, these devices are ineffective in restoring the normal biological and biomechanical function of the aorta. A stent-graft device with mechanical properties that are comparable with the aorta and aortic arch could possibly lead to fewer post-operative complications, thus, better outcome for patients with complex aneurysm conditions. This review highlights the influence stent-graft design has on the biomechanical properties of the aorta which in turn can contribute to complications of complex aneurysm repair. Design attributes critical for minimising postoperative biomechanical mismatch are also discussed.

  15. Persistent fifth arch anomalies - broadening the spectrum to include a variation of double aortic arch vascular ring

    Energy Technology Data Exchange (ETDEWEB)

    Newman, Beverley; Chan, Frandics [Stanford Children' s Hospital and Stanford University, Department of Radiology, Stanford, CA (United States); Hanneman, Kate [University of Toronto, Department of Medical Imaging, Toronto, ON (Canada)

    2016-12-15

    Fifth arch anomalies are rare and complex and frequently misdiagnosed or mistaken for other entities. We report a double arch vascular ring that is thought to consist of right fourth arch and left fifth arch components, a previously undescribed persistent fifth arch variant. The currently recognized spectrum and classification of fifth arch vascular anomalies are expanded along with illustrative images to justify the proposed changes. Reviewing and expanding the classification of fifth arch anomalies to include a double arch ring variant will promote recognition, correct diagnosis and appropriate management of these anomalies. (orig.)

  16. Pregnancy with aortic dissection in Ehler-Danlos syndrome. Staged replacement of the total aorta (10-year follow-up).

    Science.gov (United States)

    Babatasi, G; Massetti, M; Bhoyroo, S; Khayat, A

    1997-10-01

    Pregnancy complicated by aortic dissection in patients with hereditary disorder of connective tissue presents interesting considerations including management of caesarean section with the unexpected need for cardiac surgery in emergency. Generalizations can be made on management principles with long-term follow-up requiring an aggressive individualized approach by a multidisciplinary team. A 33-year-old parturient presenting an aortic dissection at 37 weeks gestation required prompt diagnosis of Ehlers-Danlos syndrome in combination with correct surgical therapy resulted in the survival of both the mother and infant. During the 10-year follow-up, multiple complex dissection required transverse aortic arch and thoracoabdominal aortic replacement.

  17. Calcification at orifices of aortic arch branches is a reliable and significant marker of stenosis at carotid bifurcation and intracranial arteries

    Energy Technology Data Exchange (ETDEWEB)

    Yamada, Shigeki, E-mail: shigekiyamada3@gmail.com [Department of Neurosurgery and Stroke Center, Rakuwakai Otowa Hospital, Kyoto (Japan); Interfaculty Initiative in Information Studies/Institute of Industrial Science, The University of Tokyo, Tokyo (Japan); Department of Neurosurgery, Hamamatsu Rosai Hospital, Shizuoka (Japan); Hashimoto, Kenji, E-mail: hashiken8022@yahoo.co.jp [Department of Neurosurgery, Kishiwada Municipal Hospital, Osaka (Japan); Ogata, Hideki, E-mail: hidogata@gmail.com [Department of Neurosurgery, Hamamatsu Rosai Hospital, Shizuoka (Japan); Watanabe, Yoshihiko, E-mail: ynabe@magic.odn.ne.jp [Department of Neurosurgery, Hamamatsu Rosai Hospital, Shizuoka (Japan); Oshima, Marie, E-mail: marie@iis.u-tokyo.ac.jp [Interfaculty Initiative in Information Studies/Institute of Industrial Science, The University of Tokyo, Tokyo (Japan); Miyake, Hidenori, E-mail: hi-miyake@hamamatsuh.rofuku.go.jp [Department of Neurosurgery, Hamamatsu Rosai Hospital, Shizuoka (Japan)

    2014-02-15

    Purpose: Simple rating scale for calcification in the cervical arteries and the aortic arch on multi-detector computed tomography angiography (MDCTA) was evaluated its reliability and validity. Additionally, we investigated where is the most representative location for evaluating the calcification risk of carotid bifurcation stenosis and atherosclerotic infarction in the overall cervical arteries covering from the aortic arch to the carotid bifurcation. Method: The aortic arch and cervical arteries among 518 patients (292 men, 226 women) were evaluated the extent of calcification using a 4-point grading scale for MDCTA. Reliability, validity and the concomitant risk with vascular stenosis and atherosclerotic infarction were assessed. Results: Calcification was most frequently observed in the aortic arch itself, the orifices from the aortic arch, and the carotid bifurcation. Compared with the bilateral carotid bifurcations, the aortic arch itself had a stronger inter-observer agreement for the calcification score (Fleiss’ kappa coefficients; 0.77), but weaker associations with stenosis and atherosclerotic infarction. Calcification at the orifices of the aortic arch branches had a stronger inter-observer agreement (0.74) and enough associations with carotid bifurcation stenosis and intracranial stenosis. In addition, the extensive calcification at the orifices from the aortic arch was significantly associated with atherosclerotic infarction, similar to the calcification at the bilateral carotid bifurcations. Conclusions: The orifices of the aortic arch branches were the novel representative location of the aortic arch and overall cervical arteries for evaluating the calcification extent. Thus, calcification at the aortic arch should be evaluated with focus on the orifices of 3 main branches.

  18. Aortic arch and intra-/extracranial cerebral arterial atherosclerosis in patients suffering acute ischemic strokes

    Institute of Scientific and Technical Information of China (English)

    郭毅; 姜昕; 陈实; 张少文; 赵宏文; 吴瑛

    2003-01-01

    Objective To determine the distribution of aortic arch and intra/extracranial cerebral arterial atherosclerosis in Chinese patients who had suffered acute ischemic strokes. Methods Eighty-nine patients with acute ischemic strokes were included in this study. Transesophageal echocardiography (TEE) was used to evaluate potential sources of embolisms in the aortic arch and in the heart; duplex ultrasound was used for the carotid artery; and intracranial Doppler (TCD) imaging was used for the middle cerebral artery (MCA), anterior cerebral artery (ACA), posterior cerebral artery (PCA), and basilar artery (BA). An atherosclerotic lesion in the aortic arch was defined as normal (0); mild plaque (1); moderate plaque (2); and protruding plaque or mobile plaque (3). A lesion in the carotid artery was considered a plaque if the maximal carotid plaque thickness was 1.2 mm. TCD results were deemed abnormal if flow velocity was either greater or lower than normal, and, in the case of the MCA, if an asymmetry index above 21% was measured. Results Of the 89 patients, 52 (58.43%) patients showed evidence of aortic arch atherosclerosis (AAA), including 11 (12.36%) patients graded mild, 18 (20.22%) patients graded moderate, and 23 (25.84%) patients graded severe. Of the 23 patients with severe AAA, AAA was determined to be an important potential embolic source in 14 patients. Forty-nine (50.56%) patients had carotid arterial plaques (CAPs). The incidence of carotid plaques was higher among patients with AAA than among patients without AAA (71.15% vs 21.62%, OR=3.291, 95% CI=1.740-6.225, P<0.001). TCD abnormalities affecting the MCA were found in 54 (60.67%) patients. Differences in incidence of TCD abnormalities between patients with AAA and without AAA (69.23% vs 48.65%) were not significant (OR=1.423, 95% CI=0.976-2.076, P=0.05). There was a higher incidence of AAA in older, male patients with a history of diabetes and smoking. Conclusions AAA is an important potential source of

  19. Isolation of the Left Innominate Artery with a Right Aortic Arch in a 76 year-old Man: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Tong; Jou, Sung Shick; Bae, Won Kyung [Soonchunhyang University Cheonan Hospital, Cheonan (Korea, Republic of)

    2009-05-15

    Isolation of the left innominate artery is a rare anomaly and is usually combined with a right side aortic arch. Most patients are neonates or children with congenital heart disease. We report the MDCT findings of a right aortic arch and isolation of the left innominate artery in a 76-year-old man without congenital heart disease

  20. Interrupção do arco aórtico tipo B em uma paciente com síndrome de olho de gato Interrupción del arco aórtico tipo B en una paciente con síndrome del ojo de gato Interrupted aortic arch type B in A patient with cat eye syndrome

    Directory of Open Access Journals (Sweden)

    Sintia Iole Nogueira Belangero

    2009-05-01

    Full Text Available Relatamos um caso de paciente com Síndrome do Olho de Gato (Cat Eye Syndrome-CES e interrupção do arco aórtico tipo B, um achado típico na síndrome da deleção 22q11.2. A análise cromossômica e a técnica de hibridização fluorescente in situ (FISH mostraram um cromossomo marcador isodicêntrico supranumerário com bi-satélite derivado do cromossomo 22. O segmento de 22pter a 22q11.2 no cromossomo supranumerário encontrado em nosso paciente não estava em sobreposição com a região deletada em pacientes com a síndrome da deleção 22q11.2. Entretanto, o achado de interrupção do arco aórtico tipo B não é usual na CES, mas é um defeito cardíaco freqüente na síndrome da deleção 22q11.Informamos un caso de paciente con Síndrome de Ojo de Gato (Cat Eye Syndrome-CES e Interrupción del Arco Aórtico tipo B, un hallazgo típico en el síndrome de la deleción 22q11.2. El análisis cromosómico y la técnica de hibridación in situ fluorescente (FISH mostraron un cromosoma marcador isodicéntrico supernumerario bisatelitado derivado del cromosoma 22. El segmento de 22pter a 22q11.2 en el cromosoma supernumerario encontrado en nuestro paciente no estaba en sobreposición con la región deletada en pacientes con el síndrome de la deleción 22q11.2. Con todo, el hallazgo de interrupción del arco aórtico tipo B no es usual en el CES, sino que es un defecto cardíaco frecuente en el síndrome de deleción 22q11.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

  1. Dopplersonographic diagnosis of subclavian steal in infants with coarctation of the aorta and interrupted aortic arch. [Comparison with angiocardiographic findings

    Energy Technology Data Exchange (ETDEWEB)

    Deeg, K.H.; Singer, H.

    1989-03-01

    In two newborns with severe coarctation of the aorta and interrupted aortic arch, subclavian-steal was shown by angiocardiography. In both children pulsed doppler recordings were obtained in the cerebral arteries: Normal forward flow during systole and diastole could be shown in the anterior cerebral arteries, both internal carotid arteries, the basilar artery and the right vertebral artery. In the left vertebral artery in both infants a negative flow indicating backflow from the brain could be shown. Pulsed doppler sonography of the flow in the vertebral arteries is a non invasive method for diagnosis of subclavian steal in infants with coarctation of the aorta and interrupted aortic arch.

  2. AORTIC ANEURYSM: A RARE CAUSE OF ORTNER’S SYNDROME

    Directory of Open Access Journals (Sweden)

    Sukanta

    2014-11-01

    Full Text Available A 60 year old male patient presented with hoarseness of voice lasting for 2 months. First he was examined by the otorhinolaryngologist of our hospital, who noticed absence of movements of the left vocal cord. Chest X-ray showed a large opacity in the left upper zone and the patient was referred to the pulmonologist. His general physical examination was unremarkable not having any stigmata of Marfan's or Ehler Danlos syndrome. On chest auscultation, a systolic flow murmur was audible and there was decreased breath sound in the left infraclavicular area. Bronchoscopy revealed absence of movement of left vocal cord. His trans-thoracic echocardiography revealed degenerative aortic and mitral valve disease with mild aortic regurgitation and mild mitral regurgitation with dilated aortic aneurysm. A contrast enhanced CT scan of thorax and CT Angio Aortogram revealed fusiform aneurysmal dilatation of the arch of aorta with diameter of 6.2 cm over a length of 12.3 cm showing intraluminal partial thrombus with wall calcification. The patient was advised to undergo surgery. However, after detailed discussion with him and his family, they decided for medical management in view of his advanced age

  3. Medical image of the week: atherosclerotic aneurysm of aortic arch and descecnding thoracic aorta

    Directory of Open Access Journals (Sweden)

    Parasram M

    2016-02-01

    Full Text Available No abstract available. Article truncated after 150 words. A 94-year-old Spanish-speaking woman presented to the hospital with intermittent episodes of dyspnea and abdominal pain for one week. Her past medical history was notable for 30 pack-year smoking history and hypertension, which was reportedly controlled with medical therapy. Physical exam showed trace peripheral edema bilaterally, intact peripheral pulses, and a mild abdominal bruit. Work up at the emergency department revealed a non-ST elevation myocardial infarction with troponin T of 0.34 ng/mL but no ST-wave abnormality on electrocardiography. Chest x-ray displayed an incidental thoracic aneurysm (Figure 1. Chest computed tomography with contrast demonstrated a continuous aneurysm of the aortic arch and descending thoracic aorta with diameters measuring 6.8 cm and 6 cm, respectively (Figure 2A and 2B. Eccentric thrombi are noted in the aortic arch and the descending aorta. Interestingly, the distal descending thoracic aorta curves as it transitions to the abdominal aorta, which is evidence of a tortuous descending ...

  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

    The aim of this study was to estimate the incidence of the post-implantation syndrome/systemic inflammatory response syndrome (SIRS) after endovascular aortic repair.......The aim of this study was to estimate the incidence of the post-implantation syndrome/systemic inflammatory response syndrome (SIRS) after endovascular aortic repair....

  5. Endovascular treatment of aortic arch aneurysms Tratamento endovascular dos aneurismas de arco aórtico

    Directory of Open Access Journals (Sweden)

    Roberto Chiesa

    2008-06-01

    Full Text Available BACKGROUND: Endovascular approach to the aortic arch is an appealing solution for selected patients. OBJECTIVE: To compare the technical and clinical success recorded in the different anatomical settings of endografting for aortic arch disease. METHODS: Between June 1999 and October 2006, among 178 patients treated at our institution for thoracic aorta disease with a stent-graft, the aortic arch was involved in 64 cases. According to the classification proposed by Ishimaru, aortic zone 0 was involved in 14 cases, zone 1 in 12 cases and zone 2 in 38 cases. A hybrid surgical procedure of supra-aortic debranching and revascularization was performed in 37 cases. RESULTS: Zone 0. Proximal neck length: 44±6 mm. Initial clinical success was 78.6%: two deaths (stroke, one type Ia endoleak. At a mean follow-up of 16.4±11 months the midterm clinical success was 85.7%. Zone 1. Proximal neck length: 28±5 mm. Initial clinical success was 66.7%: 0 deaths, four type Ia endoleaks. At a mean follow-up of 16.9±17.2 months the midterm clinical success was 75.0%. Zone 2. Proximal neck length: 30±5 mm. Initial clinical success was 84.2%: two deaths (one cardiac arrest, one multiorgan embolization, three type Ia endoleaks, one case of open conversion. Two cases of delayed transitory paraparesis/paraplegia were observed. At a mean follow-up of 28.0±17.2 months the midterm clinical success was 89.5%. CONCLUSIONS: This study and a literature review demonstrated that hybrid procedure for aortic arch pathology is feasible in selected patients at high risk for conventional surgery. Our experience is still limited by the relatively small sample size. We propose to reserve zone 1 for patients unfit for sternotomy or in cases with aortic neck length > 30 mm following left common carotid artery debranching. We recommend to perform complete aortic rerouting of the aortic arch in cases with lesser comorbidities and shorter aortic neck.CONTEXTO: O tratamento endovascular

  6. Magnetic resonance angiography of the aortic arch and its main arteries directed to the brain with use of head neck coil. With special reference to relation between high position of the aortic arch and kinked vertebral and carotid arteries

    Energy Technology Data Exchange (ETDEWEB)

    Morita, Hitoshi [Saitama Medical School, Moroyama (Japan)

    1996-12-01

    We studied 316 individuals who requested general health examinations (March 1993 to February 1995), and who underwent blood tests, urinalysis and MRA with a head neck coil, and 25 volunteers underwent the same examinations. The head neck coil used covered the head, neck and upper thorax, and each individual coil could transmit and receive signals. Three-dimensional time of flight MRA was employed in the investigation. The saturation pulse was placed in a coronal direction behind the slab to suppress the venous inflow signals. Our results indicate that in all individuals, MRA clearly demonstrated the aorta and its main branches extending up to the circle of Willis. It was possible to rotate the reconstructed 3-D image around the body axis permitting the visualization of secondary or more peripheral branches, depending on their size and direction. The severity of V1 kinking, classified by using the modified Mets criteria, correlated well with advancing age. V1 kinking was first evident in the third decade of life, while kinking of the internal carotid artery appeared in the fourth decade. The highest level of the aortic arch was evaluated in terms of the aorto-C2 (A-C2) distance, i.e., the distance measured from the C2 level, where the vertebral artery curved laterally in the transverse foramen of C2, and the A level, where the upper margin of the aortic arch was highest. Shortening of the A-C2 distance was noted with aging, and a good correlation between increased height of the aortic arch and aging was seen. As compared to conventional contrast arteriography, MRA of the aortic arch and its branch arteries extending into the cranium does provide accurate data. A high position of the aortic arch, buckling of the great vessels, and tortuosity, kinking and coiling of the vertebral and carotid arteries are clearly shown. (K.H.)

  7. Isolated left brachiocephalic artery with the right aortic arch: A rare differential of large patent ductus arteriosus

    Science.gov (United States)

    Dubey, Gajendra; Gupta, Saurabh Kumar; Kothari, Shyam Sundar

    2017-01-01

    We report a case of isolation of the left brachiocephalic artery with the right aortic arch in a 9-year-old male child masquerading as large patent ductus arteriosus with left-to-right shunt. We have emphasized the subtle clinical findings which served as clues to the diagnosis. PMID:28163435

  8. Right Aortic Arch with a Retroesophageal Left Subclavian Artery and an Anomalous Origin of the Pulmonary Artery from the Aorta

    Science.gov (United States)

    Jeon, Chang-Seok; Shim, Man-shik; Yang, Ji-Hyuk; Jun, Tae-Gook

    2017-01-01

    We report the case of a newborn with a rare anatomic variation: a right aortic arch with a retroesophageal left subclavian artery and an anomalous origin of the pulmonary artery from the aorta. This variation was diagnosed using echocardiography and computed tomography, and we treated the condition surgically. PMID:28180103

  9. First and second branchial arch syndromes: multimodality approach

    Energy Technology Data Exchange (ETDEWEB)

    Senggen, Elodie; Laswed, Tarek; Meuwly, Jean-Yves; Maestre, Leonor Alamo; Meuli, Reto; Gudinchet, Francois [University Hospital of Lausanne, Radiology Department, Lausanne (Switzerland); Jaques, Bertrand [University Hospital of Lausanne, Department of Otorhinolaryngology, Lausanne (Switzerland)

    2011-05-15

    First and second branchial arch syndromes (BAS) manifest as combined tissue deficiencies and hypoplasias of the face, external ear, middle ear and maxillary and mandibular arches. They represent the second most common craniofacial malformation after cleft lip and palate. Extended knowledge of the embryology and anatomy of each branchial arch derivative is mandatory for the diagnosis and grading of different BAS lesions and in the follow-up of postoperative patients. In recent years, many new complex surgical approaches and procedures have been designed by maxillofacial surgeons to treat extensive maxillary, mandibular and external and internal ear deformations. The purpose of this review is to evaluate the role of different imaging modalities (orthopantomogram (OPG), lateral and posteroanterior cephalometric radiographs, CT and MRI) in the diagnosis of a wide spectrum of first and second BAS, including hemifacial microsomia, mandibulofacial dysostosis, branchio-oto-renal syndrome, Pierre Robin sequence and Nager acrofacial dysostosis. Additionally, we aim to emphasize the importance of the systematic use of a multimodality imaging approach to facilitate the precise grading of these syndromes, as well as the preoperative planning of different reconstructive surgical procedures and their follow-up during treatment. (orig.)

  10. "Squid-capture" modified in situ stent-graft fenestration technique for aortic arch aneurysm repair.

    Science.gov (United States)

    Hongo, Norio; Miyamoto, Shinji; Shuto, Rieko; Wada, Tomoyuki; Kamei, Noritaka; Sato, Aiko; Matsumoto, Shunro; Kiyosue, Hiro; Mori, Hiromu

    2014-08-01

    An 83-year-old female was found to have an fusiform aneurysm in the aortic arch. She was deemed to be a high surgical risk; therefore, endovascular stent-graft placement followed by revascularization of the brachiocephalic trunk using in situ stent-graft fenestration was considered. However, the safe application of fenestration was deemed difficult due to the tortuosity of the brachiocephalic artery. The patient was successfully treated with the aid of the "squid-capture" technique, which consists of deployment of the stent-graft in a snare wire loop that was advanced from the brachiocephalic artery and fenestration of the stent-graft with the support of the loop. A follow-up exam revealed complete sealing of the aneurysm without any complications. The squid-capture technique allows for the safe and secure puncture of the graft.

  11. Aortic valve replacement for aortic stenosis with a small aortic annulus in a patient having Werner's syndrome and liver cirrhosis.

    Science.gov (United States)

    Sogawa, M; Kasuya, S; Yamamoto, K; Koshika, M; Oguma, F; Hayashi, J

    2001-12-01

    Werner's syndrome is a rare genetic disease characterized by premature aging and scleroderma-like involvement of the skin. We report a case of aortic valve replacement for severely calcified aortic valve stenosis with a small annulus in a patient suffering from Werner's syndrome and liver cirrhosis

  12. Duplo arco aórtico: a quebra do silêncio Double aortic arch: the break of silence

    Directory of Open Access Journals (Sweden)

    Ana Rita Abrão

    2011-03-01

    Full Text Available Anéis vasculares representam 1-2% dos casos das cardiopatias congênitas. Relatamos um caso raro de duplo arco aórtico. Mulher, 60 anos, procurou atendimento na clínica médica apresentando 1 ano de história de disfagia, 6 meses de dispneia e 2 meses de dor torácica esporádica. Raio X de tórax revelou: hiperinsuflação pulmonar difusa, alargamento mediastinal, coração com volume e configurações normais, arco aórtico à direita e alterações degenerativas vertebrais. Tomografia computadorizada do tórax: arco aórtico duplo circundando e comprimindo a traqueia e o esôfago. Arco direito mais calibroso, emergindo dele o tronco braquiocefálico. Do arco esquerdo emergem a artéria carótida comum e a subclávia esquerda. Diagnóstico: anel vascular traqueoesofagiano decorrente do duplo arco aórtico, sendo o arco direito dominante. No presente caso, optou-se por seguimento clínico da paciente, levando-se em conta a intensidade dos sintomas apresentados.Vascular rings represent 1 to 2% of cases of congenital heart disease. We report a rare case of double aortic arch. A 60-year-old woman was admitted to the hospital presenting a one-year history of dysphagia, six months of dyspnea and two months of sporadic chest pain. Radiograph of the chest revealed diffuse pulmonary hyper inflation, widening of the mediastinum, heart of normal size and shape, a right-sized aortic arch, and degenerative changes of the thoracic spine. Computed tomography of the chest showed a double aortic arch encircling and compressing the trachea and the esophagus. The right aortic arch had a larger caliber, with brachiocephalic trunk arising from it. The left common carotid artery and the left subclavian artery arose from the left aortic arch. Diagnosis: tracheoesophageal vascular ring due to double aortic arch, with dominant right arch. In this case, we chose to follow the patient medically, taking into consideration the mildness of the symptoms.

  13. Sustained maternal hyperoxygenation improves aortic arch dimensions in fetuses with coarctation

    Science.gov (United States)

    Zeng, Shi; Zhou, Jiawei; Peng, Qinghai; Deng, Wen; Zhang, Ming; Zhao, Yili; Wang, Tao; Zhou, Qichang

    2016-01-01

    The aim was to investigate the impact of maternal hyperoxygenation (HO) on cardiac dimensions in fetuses with isolated Coarctation (CoA). Fetal echocardiography was performed serially in 48 fetuses with CoA and gestation age matched normal fetues. The Z-scores for the mitral valve (MV), tricuspid valve (TV), aortic valve (AV), ascending aorta (AAo), isthmus, pulmonary valve (PV), main pulmonary artery (MPA), and descending aorta (DAo) were measured and compared among normal fetuses, CoA fetuses with oxygen and CoA fetuses with air. In the group with oxygen, 6 L/min oxygen was administered to the mother using a face mask. Regression analyses were performed to identify potential factors for HO outcome. The left heart dimension Z-scores increased gradually during HO therapy periods, especially at 4 weeks after oxygen therapy (P < 0.05). As for the case group with air, the left heart dimension remained unchanged. The duration of HO was associated with aortic arch Z-scores (adjusted R2 = 0.199, 0.60 for AAO and isthmus, respectively). Sustained maternal middle-flow oxygenation can be safely used to improve left heart dimensions in fetuses with isolated CoA. The duration of HO were associated with treatment outcome. These findings may provide useful information for developing novel treatment strategies. PMID:27982102

  14. Thoracic type Ia endoleak: direct percutaneous coil embolization of the aortic arch at the blood entry site after TEVAR and double-chimney stent-grafts

    Energy Technology Data Exchange (ETDEWEB)

    Bangard, Christopher; Franke, Mareike; Maintz, David; Chang, De-Hua [University Hospital, University of Cologne, Department of Radiology, Cologne (Germany); Pfister, Roman [University Hospital, University of Cologne, Department of Internal Medicine III, Cologne (Germany); Deppe, Antje-Christin [University Hospital, University of Cologne, Department of Cardiothoracic Surgery, Cologne (Germany); Matoussevitch, Vladimir [University Hospital, University of Cologne, Department of Vascular Surgery, Cologne (Germany)

    2014-06-15

    To introduce a novel percutaneous technique to stop blood entry at the lesser aortic arch curvature by coil embolisation in type Ia endoleak after TEVAR. A 61-year-old Marfan patient presented with type Ia endoleak of the aortic arch and a growing aortic arch pseudoaneurysm after TEVAR. Multiple preceding operations and interventions made an endovascular approach unsuccessful. Direct percutaneous puncture of the aneurysmal sac would have cured the sign, but not the cause of blood entry at the lesser curvature of the aortic arch. Direct CT-guided percutaneous puncture of the blood entry site in the aortic arch with fluoroscopically guided coil embolisation using detachable extra-long coils was successfully performed. Three weeks after the intervention, the patient developed fever because of superinfection of the pseudoaneurysm. The blood cultures and CT-guided mediastinal aspirate were sterile. After intravenous administration of antibiotics, the fever disappeared and the patient recovered. Six-month follow-up showed permanent closure of the endoleak and a shrinking aneurysmal sac. Direct percutaneous puncture of the aortic arch at the blood entry site of a thoracic type Ia endoleak after TEVAR and double-chimney stent-grafts with coil embolisation of the wedge-shaped space between the lesser aortic curvature and the stent-graft is possible. (orig.)

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

    NARCIS (Netherlands)

    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

    2015-01-01

    BACKGROUND: Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome. OBJECTIVES: 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 dissect

  16. Spectrum of Aortic Valve Abnormalities Associated with Aortic Dilation Across Age Groups in Turner Syndrome

    Science.gov (United States)

    Olivieri, Laura J.; Baba, Ridhwan Y.; Arai, Andrew E.; Bandettini, W. Patricia; Rosing, Douglas R.; Bakalov, Vladimir; Sachdev, Vandana; Bondy, Carolyn A.

    2014-01-01

    Background Congenital aortic valve fusion is associated with aortic dilation, aneurysm and rupture in girls and women with Turner syndrome (TS). Our objective was to characterize aortic valve structure in subjects with TS, and determine the prevalence of aortic dilation and valve dysfunction associated with different types of aortic valves. Methods and Results The aortic valve and thoracic aorta were characterized by cardiovascular magnetic resonance imaging in 208 subjects with TS in an IRB-approved natural history study. Echocardiography was used to measure peak velocities across the aortic valve, and the degree of aortic regurgitation. Four distinct valve morphologies were identified: tricuspid aortic valve (TAV) 64%(n=133), partially fused aortic valve (PF) 12%(n=25), bicuspid aortic valve (BAV) 23%(n=47), and unicuspid aortic valve (UAV) 1%(n=3). Age and body surface area (BSA) were similar in the 4 valve morphology groups. There was a significant trend, independent of age, towards larger BSA-indexed ascending aortic diameters (AADi) with increasing valve fusion. AADi were (mean +/− SD) 16.9 +/− 3.3 mm/m2, 18.3 +/− 3.3 mm/m2, and 19.8 +/− 3.9 mm/m2 (p<0.0001) for TAV, PF and BAV+UAV respectively. PF, BAV, and UAV were significantly associated with mild aortic regurgitation and elevated peak velocities across the aortic valve. Conclusions Aortic valve abnormalities in TS occur with a spectrum of severity, and are associated with aortic root dilation across age groups. Partial fusion of the aortic valve, traditionally regarded as an acquired valve problem, had an equal age distribution and was associated with an increased AADi. PMID:24084490

  17. Acute aortic syndromes: Role of multi-detector row CT

    Energy Technology Data Exchange (ETDEWEB)

    Salvolini, Luca; Renda, Pietro [Department of Radiology, University ' Politecnica delle Marche' , Ancona (Italy); Fiore, Davide [Department of Radiology, University of Padova, Padova (Italy); Scaglione, Mariano [Emergency and Trauma CT Section, Department of Radiology, Cardarelli Hospital, Naples (Italy); Piccoli, GianPiero [Cardiosurgery Division, Ospedali Riuniti ' Torrette-Lancisi-Salesi' , Ancona (Italy); Giovagnoni, Andrea [Department of Radiology, University ' Politecnica delle Marche' , Ancona (Italy)], E-mail: a.giovagnoni@univpm.it

    2008-03-15

    Acute thoracic aortic syndromes encompass a spectrum of emergencies including aortic dissection, intramural haematoma, penetrating atherosclerotic ulcer and aneurysm rupture. All these life-threatening conditions require prompt diagnosis and appropriate management. To date multi-detector row Computed Tomography represents a valuable diagnostic tool especially in the emergency setting. This paper focus on the use of multi-detector row Computed Tomography in the evaluation of acute thoracic aortic syndromes and illustrates the key imaging findings related to each disease.

  18. CT Virtual angioscopy of aortic arch%CT仿真血管内镜观察主动脉弓

    Institute of Scientific and Technical Information of China (English)

    李新平; 陈伟; 朱智明

    2011-01-01

    [ Objective ] To explore the clinical value of CT virtual angioscopy (CTVA) in the pre- and postpro-cedure evaluation of aortic arch related diseases. [Methods] 37 cases with thoracic disease underwent aortic CT angiography on a dual-source CT scanner. CTVA of aortic arch were performed with Fly-through software in all cas-? Es by using aortic CT angiographic source image data. [Results] 34 of 37 (92%) cases showed successful CTVA images, including aortic dissection (n =12), aneurysm (n =4), pseudoaneurysm (n =3), Takayasu arteritis (n =2),aortic coarctation (n =1), aneurysm combined with aortic dissection (n =1) and 11 patients following surgical or endovascu-lar procedures. CTVA of aortic arch could identify the ostia and its variations of supra-aortic vessels, estimate the localization of abnormalities with respect to supra-aortic vessels and display the stent grafts. [ Conclusions ] CTVA of aortic arch enables non-invasive visualization of the inner contours of the vasculature, and has certain instructive significance in the diagnostic classification, preprocedure planning and postprocedure evaluation for aortic arch related diseases.%目的 探讨CT仿真血管内镜(CTVA)在主动脉弓相关疾病术前和术后评价中的价值.方法 采用双源CT对37例胸主动脉疾病患者行主动脉CT成像检查,并将主动脉CT成像的源图像调至Fly-through软件中,对所有病例行主动脉弓CTVA观察.结果 92%(34/37)的病例获得了满意的CTVA图像,包括主动脉夹层12例、真性动脉瘤4例、假性动脉瘤3例、多发性大动脉炎2例、主动脉缩窄1例、真性动脉瘤合并夹层1例及11例术后随访病例.主动脉弓CTVA能明确弓上分支血管开口位置与变异、判断病变与弓上分支开口的空间关系以及显示支架型血管.结论 主动脉弓CTVA能无创观察血管腔内情况,对主动脉弓相关疾病的诊断分型、术前计划和术后评价具有一定的指导意义.

  19. 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...

  20. Aberrant right vertebral artery originating from the aortic arch distal to the left subclavian artery: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Baek, Soo Heui; Baek, Hye Jin [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2014-03-15

    We present a rare case of an aberrant right vertebral artery originated from the distal aortic arch. This issue has been incidentally detected on a preoperative CT angiography after a stabbing injury of the cervical spinal cord. Normally, the right vertebral artery originates from the right subclavian artery. Therefore, in this case report we will review the incidence and the embryological mechanism of this aberrant course of the right vertebral artery and we will discuss as well the clinical importance of this variation.

  1. Correlation of atherosclerotic changes in peripheral arteries with pathological involvement of aortic arch in coronary bypass patients

    Directory of Open Access Journals (Sweden)

    Eshraghi N

    2010-10-01

    Full Text Available "nBackground: A correlation between coronary artery disease (CAD and atherosclerosis of peripheral arteries and the determination of noninvasive indexes for its existence and extent have been sought by many researchers. Some studies report that the intima-media thickness (IMT of peripheral arteries could play this role. This study evaluated the correlation between the IMTs of common carotid and common femoral arteries and the degree of atherosclerosis in aortic arch and to evaluate the severity of CAD in candidates of coronary artery bypass grafting (CABG."n "nMethods: In a cross-sectional analytic-descriptive study, The severity of CAD, the grade of atherosclerosis of the aortic arch, and the IMTs of the common carotid and common femoral arteries were determined."n "nResults: There was a significant weak positive correlation between the IMT of common carotid artery (ρ = 0.193, p = 0.039 and common femoral artery (ρ = 0.206, p = 0.028 with the number of involved carotid vessels; the mean of these two parameters was not significantly different between the three CAD groups. There was not any significant relation between the IMTs of common carotid and common femoral arteries with the severity of atherosclerosis in the aortic arch too. There was not any significant relation between the presences of atherosclerotic plaque in the common carotid or the common femoral arteries with the severity of CAD. The severe atherosclerosis of the aortic arch was significantly higher in patients with three vessel disease."n "nConclusion: According to our results, the IMTs of common carotid and/or common femoral arteries may increase with the severity of CAD; however, these parameters are not a surrogate for predicting the CAD severity.

  2. Pulmonary artery stenosis caused by a large aortic arch pseudoaneurysm detected 10 years after a minor trauma

    Directory of Open Access Journals (Sweden)

    Jalal Zamani

    2016-03-01

    Full Text Available Pseudoaneurysm of aorta is a rare condition usually seen after aortic surgeries or serious accidents. Here we report a 60 years old man without any previous medical condition who presented with non-specific symptoms and underwent different investigations for more than 1 year, until the presence of a continuous murmur raised suspicion toward his cardiovascular system. In echocardiographic and computed tomography (CT angiographic studies a large pseudoaneurysm of aortic arch with compression effect on pulmonary artery was detected. At this stage he remembered having suffered a minor trauma 10 years ago. He finally underwent operation and his aortic wall was repaired successfully with a patch. This case highlights the importance of thorough history taking and physical examination in patients irrespective of symptoms and high index of suspicion to detect this life-threatening condition.

  3. Bicuspid aortic valve and aortic coarctation are linked to deletion of the X chromosome short arm in Turner syndrome

    Science.gov (United States)

    Bondy, Carolyn; Bakalov, Vladimir K; Cheng, Clara; Olivieri, Laura; Rosing, Douglas R; Arai, Andrew E

    2013-01-01

    Background Congenital heart disease (CHD) is a cardinal feature of X chromosome monosomy, or Turner syndrome (TS). Haploinsufficiency for gene(s) located on Xp have been implicated in the short stature characteristic of the syndrome, but the chromosomal region related to the CHD phenotype has not been established. Design We used cardiac MRI to diagnose cardiovascular abnormalities in four non-mosaic karyotype groups based on 50-metaphase analyses: 45,X (n=152); 46,X,del(Xp) (n=15); 46,X,del(Xq) (n=4); and 46,X,i(Xq) (n=14) from peripheral blood cells. Results Bicuspid aortic valves (BAV) were found in 52/152 (34%) 45,X study subjects and aortic coarctation (COA) in 19/152 (12.5%). Isolated anomalous pulmonary veins (APV) were detected in 15/152 (10%) for the 45,X study group, and this defect was not correlated with the presence of BAV or COA. BAVs were present in 28.6% of subjects with Xp deletions and COA in 6.7%. APV were not found in subjects with Xp deletions. The most distal break associated with the BAV/COA trait was at cytologic band Xp11.4 and ChrX:41,500 000. One of 14 subjects (7%) with the 46,X,i(Xq) karyotype had a BAV and no cases of COA or APV were found in this group. No cardiovascular defects were found among four patients with Xq deletions. Conclusions The high prevalence of BAV and COA in subjects missing only the X chromosome short arm indicates that haploinsufficiency for Xp genes contributes to abnormal aortic valve and aortic arch development in TS. PMID:23825392

  4. 主动脉缩窄或主动脉弓中断合并心内畸形一期修复%One-stage repair of aortic coarctation or interrupted aortic arch associated with cardiac anomalies through median sternotomy

    Institute of Scientific and Technical Information of China (English)

    方敏华; 朱洪玉; 汪曾炜; 王辉山; 李新民; 宋恒昌

    2010-01-01

    was one postoperative death.The causs was pulmonary hypertension and severe low cardiac output syndrome.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 6,pneumonia in 11,atelectasis in 14,injury of recurrent laryngeal nerve in 19,and supra ventricular tachycardia in 23.34 patients were followed up from 3 months to 5 years and were in good condition without recoarctation.Conclusion The outcomes of early and medium term for one-stage repair of aortic coarctation or interrupted aortic arch and associated cardiac anomalies through median sternotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation

  5. Hybrid Repair of Complex Thoracic Aortic Arch Pathology: Long-Term Outcomes of Extra-anatomic Bypass Grafting of the Supra-aortic Trunk

    Energy Technology Data Exchange (ETDEWEB)

    Lotfi, S., E-mail: shamim.lotfi@kcl.ac.uk; Clough, R. E.; Ali, T. [Guy' s and St. Thomas' NHS Trust, Vascular Surgery (United Kingdom); Salter, R. [Guy' s and St. Thomas' NHS Trust, Interventional Radiology (United Kingdom); Young, C. P. [Guy' s and St. Thomas' NHS Trust, Cardiac Surgery (United Kingdom); Bell, R.; Modarai, B.; Taylor, P., E-mail: peter.taylor@gstt.nhs.uk [Guy' s and St. Thomas' NHS Trust, Vascular Surgery (United Kingdom)

    2013-02-15

    Hybrid repair constitutes supra-aortic debranching before thoracic endovascular aortic repair (TEVAR). It offers improved short-term outcome compared with open surgery; however, longer-term studies are required to assess patient outcomes and patency of the extra-anatomic bypass grafts. A prospectively maintained database of 380 elective and urgent patients who had undergone TEVAR (1997-2011) was analyzed retrospectively. Fifty-one patients (34 males; 17 females) underwent hybrid repair. Median age was 71 (range, 18-90) years with mean follow-up of 15 (range, 0-61) months. Perioperative complications included death: 10 % (5/51), stroke: 12 % (6/51), paraplegia: 6 % (3/51), endoleak: 16 % (8/51), rupture: 4 % (2/51), upper-limb ischemia: 2 % (1/51), bypass graft occlusion: 4 % (2/51), and cardiopulmonary complications in 14 % (7/51). Three patients (6 %) required emergency intervention for retrograde dissection: (2 aortic root repairs; 2 innominate stents). Early reintervention was performed for type 1 endoleak in two patients (2 proximal cuff extensions). One patient underwent innominate stenting and revision of their bypass for symptomatic restenosis. At 48 months, survival was 73 %. Endoleak was detected in three (6 %) patients (type 1 = 2; type 2 = 1) requiring debranching with proximal stent graft (n = 2) and proximal extension cuff (n = 1). One patient had a fatal rupture of a mycotic aneurysm and two arch aneurysms expanded. No bypass graft occluded after the perioperative period. Hybrid operations to treat aortic arch disease can be performed with results comparable to open surgery. The longer-term outcomes demonstrate low rates of reintervention and high rates of graft patency.

  6. 主动脉弓部手术的护理体会%Nursing experience of aortic arch operation

    Institute of Scientific and Technical Information of China (English)

    杨爱娣

    2012-01-01

    Objective To summarize the nursing experience of aortic arch operation. Methods 27 patients underwent aortic arch operation from Jan 2007 to Apr 2012 were enrolled to the study. Clinical datum were collected and analyse the work points of operation. Results 5 cases dead, abandoning treatment in 1 case, 18 cases survived after operation (including 1 case underwent re-operation, 2 cases repeatedly hospitalized due to internal fistula and hemoglobinuria) , 1 case need life care. The dead cases have nothing to do with nursing. Conclusion Aortic arch operation requires deep hypothermic circulatory arrest, lasts longer, with higher mortality, so for nursing staff in operation room, it need more theoretical basis, skilled cooperation and flexibility.%目的 总结主动脉弓部手术的护理体会.方法 回顾性选取我院2007年1月~2012年4月所行的主动脉弓部手术27例,收集相关临床资料,分析术前、术中,巡回、器械护士的工作要点.结果 27例手术均成功实施.结论 主动脉弓部手术需要深低温停循环,手术时间长,病死率高,需要手术室护理人员更扎实的理论基础,更熟练的配合,更灵活的应变能力.

  7. Senile Calcification of the Trachea, Aortic Arch, and Mitral Annulus: An Incidental Finding on Chest X-Ray.

    Science.gov (United States)

    Hosseinzadeh Maleki, Mahmood; Kazemi, Toba; Davoody, Navid

    2015-10-27

    A 94-year-old woman presented with dizziness and hypotension of 2 days' duration. She denied any syncope, presyncope, or angina. She had received a permanent pacemaker 12 years previously for the management of complete heart block (CHB), but she failed to program it. Twelve-lead electrocardiography revealed CHB with ventricular escape rhythm (40/min), so we inserted a temporary pacemaker. Anteroposterior chest X-ray showed trachea, aortic arch, and severe mitral valve calcification. Tracheal calcification is usually seen after 40 years old without clinical importance. However, it is seen in patients with renal failure, metastases, and prolonged use of warfarin as well as in pregnancy.(1) (-) (3).

  8. Fabrication of a compliant phantom of the human aortic arch for use in Particle Image Velocimetry (PIV experimentation

    Directory of Open Access Journals (Sweden)

    Hütter Larissa

    2016-09-01

    Full Text Available Compliant phantoms of the human aortic arch can mimic patient specific cardiovascular dysfunctions in vitro. Hence, phantoms may enable elucidation of haemodynamic disturbances caused by aortic dysfunction. This paper describes the fabrication of a thin-walled silicone phantom of the human ascending aorta and brachiocephalic artery. The model geometry was determined via a meta-analysis and modelled in SolidWorks before 3D printing. The solid model surface was smoothed and scanned with a 3D scanner. An offset outer mould was milled from Ebalta S-Model board. The final phantom indicated that ABS was a suitable material for the internal model, the Ebalta S-Model board yielded a rough external surface. Co-location of the moulds during silicone pour was insufficient to enable consistent wall thickness. The resulting phantom was free of air bubbles but did not have the desired wall thickness consistency.

  9. 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.

  10. Fluid dynamics of aortic root dilation in Marfan syndrome

    CERN Document Server

    Querzoli, Giorgio; Espa, Stefania; Costantini, Martina; Sorgini, Francesca

    2014-01-01

    Aortic root dilation and propensity to dissection are typical manifestations of the Marfan Syndrome (MS), a genetic defect leading to the degeneration of the elastic fibres. Dilation affects the structure of the flow and, in turn, altered flow may play a role in vessel dilation, generation of aneurysms, and dissection. The aim of the present work is the investigation in-vitro of the fluid dynamic modifications occurring as a consequence of the morphological changes typically induced in the aortic root by MS. A mock-loop reproducing the left ventricle outflow tract and the aortic root was used to measure time resolved velocity maps on a longitudinal symmetry plane of the aortic root. Two dilated model aortas, designed to resemble morphological characteristics typically observed in MS patients, have been compared to a reference, healthy geometry. The aortic model was designed to quantitatively reproduce the change of aortic distensibility caused by MS. Results demonstrate that vorticity released from the valve ...

  11. Single-Stage Repair of an Unusual Association: Congenital Gerbode Defect, Hypoplastic Aortic Arch, and Partially Anomalous Pulmonary Venous Return in an Infant.

    Science.gov (United States)

    Flores, Saul; Kimball, Thomas R; Nelson, David P; Morales, David L S

    2016-07-01

    We present the case of a two-month-old male with congenital Gerbode defect, hypoplastic aortic arch, and left-sided partially anomalous pulmonary venous return. The patient underwent single-stage surgical repair, which consisted of aortic arch advancement with resection of the coarctation segment, pulmonary vein repair, and primary closure of the Gerbode defect. The anomalous pulmonary vein posed a particular challenge due to its size and distance from the left atrium, which we approached with a posterior atrial wall trapdoor baffle technique, without mobilizing the affected vein. Postoperatively and at one year follow-up, there was no evidence of residual lesions and there was unobstructed flow pattern across the aortic arch and the affected pulmonary vein.

  12. 4-D MRI flow analysis in the course of interrupted aortic arch reveals complex morphology and quantifies amount of collateral blood flow

    Energy Technology Data Exchange (ETDEWEB)

    Hirtler, Daniel [University Hospital Freiburg, Department of Pediatric Cardiology and Congenital Heart Disease, Freiburg (Germany); Geiger, Julia; Jung, Bernd [University Hospital Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Markl, Michael [Northwestern University, Departments of Radiology and Biomedical Engineering, Chicago, IL (United States); Arnold, Raoul [University Hospital Heidelberg, Department of Pediatric Cardiology and Congenital Heart Disease, Heidelberg (Germany)

    2013-08-15

    We present findings in a 17-year-old with interrupted aortic arch, in whom standard imaging techniques missed functional and morphological problems. Flow-sensitive four-dimensional magnetic resonance (4-D MR) enabled assessment of the complex anatomy and blood-flow characteristics in the entire aorta and direct quantification of blood flow in collateral vessels. Our findings highlight the entire morphological and functional problem of interrupted aortic arch and illustrate the potential of flow-sensitive 4-D MR for surgical planning in congenital heart disease. (orig.)

  13. Analysis of early and long-term outcomes of acute type A aortic dissection according to the new international aortic arch surgery study group recommendations.

    Science.gov (United States)

    Colli, Andrea; Carrozzini, Massimiliano; Galuppo, Marco; Comisso, Marina; Toto, Francesca; Gregori, Dario; Gerosa, Gino

    2016-10-01

    To evaluate predictors of early and long-term outcomes of surgical repair of acute Type A aortic dissection. Retrospective single-centre study evaluating patients surgically treated between 1998 and 2013. Clinical follow-up was performed. Complications were classified according to the International Aortic Arch Surgery Study Group recommendations. Statistical analysis included univariate and multivariate analysis of preoperative and operative data. One hundred eighty-five patients were evaluated. The follow-up was complete for 180 patients (97 %). Mean age was 63 years, 82 % had a DeBakey type I aortic dissection, 18 % a type II. Eleven patients (6 %) died intraoperatively, 119 of the remaining (68 %) had postoperative complications. Thirty-day mortality was 21 % (38 patients). Average ICU and hospital stay were 6 and 14 days, respectively. During a mean follow-up time of 6 ± 4 years we observed 44 deaths (31 %). Twenty patients (14 %) needed late thoracic aorta reoperation. Results from the multivariate analysis are as follows. Thirty-day mortality was associated with abdominal pain at presentation (p < 0.01). The incidence of postoperative complications was related to older age at intervention (p < 0.01) and longer cross-clamp time (p < 0.01). Mortality at follow-up was significantly increased by older age at intervention (p < 0.01), with a logarithmic growth after 60 years, female sex (p < 0.01), preoperative limb ischemia (p = 0.02) and DHCA (p < 0.01). The surgical results of type A aortic dissection are affected by age at intervention with a logarithmic increase of late mortality in patients older than 60 years.

  14. Diagnosis of aortic coarctation combined with hypoplastic aortic arch by echocardiography%超声心动图诊断主动脉缩窄合并主动脉弓发育不良的价值

    Institute of Scientific and Technical Information of China (English)

    郑春华; 王春燕; 邓风平; 刘湘君; 唐秀杰; 潘广玉; 李洪银; 吴清玉

    2010-01-01

    Objective To study the diagnosis accuracy and features of echocardiography on aortic coarctation combined with hypoplastic aortic arch. Methods The echocardiographic characteristics of seven patients who were diagnosed as aortic coarctation combined with hypoplastic aortic arch were analyzed and compared with results of cardiac catheterization, CT scan and operation. The diagnostic features of echocardiography were summarized. Results Besides the echocardiographic characteristics of aortic coarctation,other specific echocardiographic characteristics of hypoplastic aortic arch were:distinct stenoses of transverse and decsending part of aortic arch were visualized in supra-sternal long-axis view; the range of hypoplastic part started from innominate artery beginning or left common carotid artery beginning; the ratio of diameter of hypoplastic aortic arch over that of descending aorta at diaphragm was less than 0. 5; the blood flow speed detected by Doppler at the site of aortic coarctation was in the normal range. The echocardiographic characteristics of the aortic coarctation combined with hypoplastic aortic arch were correspondent to the results of cardiac catheterization, CT scan and operation results. Conclusions There is high accuracy of echocardiography in diagnosis of aortic coarctation combined with hypoplastic aortic arch. Hypoplastic aortic arch should be paid more attention in diagnosis of aortic coarctation.%目的 探讨超声心动图诊断主动脉缩窄合并主动脉弓发育不良的准确性.方法 分析7例主动脉缩窄合并主动脉弓发育不良患儿的二维超声图像,并与心血管造影,心脏CT及手术结果对比分析,总结其诊断要点.结果 除主动脉缩窄的超声表现外,超声特异表现为:胸骨上凹主动脉弓长轴切面可见主动脉横弓部及弓降部明显变窄.发育不良的范围可自无名动脉起始后或左颈总动脉起始后;发育不良主动脉弓的内径/膈肌

  15. Technical and Clinical Success and Long-Term Durability of Endovascular Treatment for Atherosclerotic Aortic Arch Branch Origin Obstruction : Evaluation of 144 Procedures

    NARCIS (Netherlands)

    van de Weijer, M. A. J.; Vonken, E. J. P. A.; de Vries, J-P. P. M.; Moll, F. L.; Vos, J. A.; de Borst, G. J.

    2015-01-01

    Objectives: Endovascular treatment of atherosclerotic obstruction of aortic arch branch origins (AABO) has largely replaced open surgery, but long-term outcome data are lacking. This study evaluated mid-term and long-term results of these procedures. Design: Retrospective cohort study. Materials and

  16. Ramos do arco aórtico de bubalinos Branches of the aortic arch of buffaloes

    Directory of Open Access Journals (Sweden)

    Leandro Marcel Fernandes Cortellini

    2000-06-01

    Full Text Available Os ramos do arco aórtico (Arcus aortae em bubalinos foram investigados neste trabalho. Assim, foram dissecadas as artérias oriundas desse arco previamente injetadas com solução corada de látex Neoprene 650â (Du Pont do Brasil S.A. em 20 fetos dessa espécie, machos e fêmeas com idades entre 4 e 8 meses de gestação. Em 80% dos casos, observou-se que o tronco braquiocefálico (Truncus brachiocephalicus emite a artéria subclávia (Arteria subclavia esquerda, artérias carótidas comuns (Arteria carotis communis esquerda e direita, sem caracterizar tronco bicarotídeo (Truncus bicaroticus, e a artéria subclávia direita. As artérias subclávias direita e esquerda originam em comum o tronco costocervical (Truncus costocervicalis, a artéria cervical superficial (Arteria cervicalis superficialis, artérias axilares (Arteria axillaris e artéria torácica interna (Arteria thoracica interna. Em 20% dos casos, o tronco braquiocefálico origina a artéria subclávia esquerda em comum ao tronco costocervical esquerdo; em seguida, emite a artéria carótida comum esquerda e termina trifurcando-se em artéria carótida comum direita, artéria subclávia direita e tronco costocervical direito, sendo que as artérias subclávias direita e esquerda têm origem comum com as artérias cervical superficial, axilar e torácica interna, com a presença do tronco bicarotídeo, característico dos bovinos.The branches of the aortic arch (Arcus aortae of buffaoes were investigated in this study. Therefore, dissections were procedeed in previously injected arteries (using coloured Neoprene latex 650â - Du Pont do Brasil S.A. of 20 buffalo foetuses, theses males and females between four and eight months of gestation . In 80% of the cases it was observed that the brachiocephalic trunk (Truncus brachiocephalicus gives off the left subclavian artery, (Arteria subclavia the right and left common carotid arteries (Arteria carotis communis - being absent the

  17. Abdominal aortic thrombosis in a patient with nephrotic syndrome.

    Science.gov (United States)

    Nakamura, M; Ohnishi, T; Okamoto, S; Yamakado, T; Isaka, N; Nakano, T

    1998-01-01

    We report a patient who presented with severe nephrotic syndrome complicated with infrarenal aortic and right renal arterial thrombosis. The nephrotic syndrome frequently causes thromboembolic complications in veins, but arterial thrombosis is relatively rare, especially in the aorta. Various predisposing factors leading to thromboembolic complications are discussed. In this case, the thromboembolic complication may have some clinical association with the hypercoagulable state in nephrotic syndrome.

  18. Aortic arch replacement with a beating heart: a simple method using continuous 3-way perfusion.

    Science.gov (United States)

    Abu-Omar, Y; Ali, J M; Colah, S; Dunning, J J

    2014-01-01

    We describe a simplified 3-way perfusion strategy that could be used in complex aortic procedures, which ensures continuous end-organ perfusion and minimizes the potential risks of cardiac, cerebral and peripheral ischaemic complications.

  19. Left thoracoscopic two-stage repair of tracheoesophageal fistula with a right aortic arch and a vascular ring

    Science.gov (United States)

    Oshima, Kazuo; Uchida, Hiroo; Tainaka, Takahisa; Tanano, Akihide; Shirota, Chiyoe; Yokota, Kazuki; Murase, Naruhiko; Shirotsuki, Ryo; Chiba, Kosuke; Hinoki, Akinari

    2017-01-01

    A right aortic arch (RAA) is found in 5% of neonates with tracheoesophageal fistulae (TEF) and may be associated with vascular rings. Oesophageal repairs for TEF with an RAA via the right chest often pose surgical difficulties. We report for the first time in the world a successful two-stage repair by left-sided thoracoscope for TEF with an RAA and a vascular ring. We switched from right to left thoracoscopy after finding an RAA. A proximal oesophageal pouch was hemmed into the vascular ring; therefore, we selected a two-stage repair. The TEF was resected and simple internal traction was placed into the oesophagus at the first stage. Detailed examination showed the patent ductus arteriosus (PDA) completing a vascular ring. The subsequent primary oesophago-oesophagostomy and dissection of PDA was performed by left-sided thoracoscope. Therefore, left thoracoscopic repair is safe and feasible for treating TEF with an RAA and a vascular ring. PMID:27143697

  20. Monozygotic twins with Marfan's syndrome and ascending aortic aneurysm.

    Science.gov (United States)

    Redruello, Héctor Jorge; Cianciulli, Tomas Francisco; Rostello, Eduardo Fernandez; Recalde, Barbara; Lax, Jorge Alberto; Picone, Victorio Próspero; Belforte, Sandro Mario; Prezioso, Horacio Alberto

    2007-08-01

    Marfan's syndrome is a hereditary connective tissue disease, in which cardiovascular abnormalities (especially aortic root dilatation) are the most important cause of morbidity and mortality. In this report, we describe two 24-year-old twins, with a history of surgery for lens subluxation and severe cardiovascular manifestations secondary to Marfan's syndrome. One of the twins suffered a type A aortic dissection, which required replacement of the ascending aorta, and the other twin had an aneurysmal dilatation of the ascending aorta (46mm) and was prescribed medical treatment with atenolol and periodic controls to detect the presence of a critical diameter (50mm) that would indicate the need for prophylactic surgery.

  1. Interdependencies of aortic arch secondary flow patterns, geometry, and age analysed by 4-dimensional phase contrast magnetic resonance imaging at 3 Tesla

    Energy Technology Data Exchange (ETDEWEB)

    Frydrychowicz, Alex [University Hospital Schleswig-Holstein, Clinic for Radiology and Nuclear Medicine, Luebeck (Germany); Berger, Alexander; Russe, Maximilian F.; Bock, Jelena [University Hospital Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Munoz del Rio, Alejandro [University of Wisconsin - Madison, Departments of Radiology and Medical Physics, Madison, WI (United States); Harloff, Andreas [University Hospital Freiburg, Department of Neurology and Clinical Neurophysiology, Freiburg (Germany); Markl, Michael [University Hospital Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Northwestern University, Departments of Radiology and Biomedical Engineering, Chicago, IL (United States)

    2012-05-15

    It was the aim to analyse the impact of age, aortic arch geometry, and size on secondary flow patterns such as helix and vortex flow derived from flow-sensitive magnetic resonance imaging (4D PC-MRI). 62 subjects (age range = 20-80 years) without circumscribed pathologies of the thoracic aorta (ascending aortic (AAo) diameter: 3.2 {+-} 0.6 cm [range 2.2-5.1]) were examined by 4D PC-MRI after IRB-approval and written informed consent. Blood flow visualisation based on streamlines and time-resolved 3D particle traces was performed. Aortic diameter, shape (gothic, crook-shaped, cubic), angle, and age were correlated with existence and extent of secondary flow patterns (helicity, vortices); statistical modelling was performed. Helical flow was the typical pattern in standard crook-shaped aortic arches. With altered shapes and increasing age, helicity was less common. AAo diameter and age had the highest correlation (r = 0.69 and 0.68, respectively) with number of detected vortices. None of the other arch geometric or demographic variables (for all, P {>=} 0.177) improved statistical modelling. Substantially different secondary flow patterns can be observed in the normal thoracic aorta. Age and the AAo diameter were the parameters correlating best with presence and amount of vortices. Findings underline the importance of age- and geometry-matched control groups for haemodynamic studies. (orig.)

  2. Nonoperative management of pediatric aortic injury with seat belt syndrome.

    Science.gov (United States)

    Parrish, Dan W; Barnhorst, Amanda; Trebska-McGowan, Katarzyna; Amendola, Michael; Haynes, Jeffrey H

    2015-08-01

    "Seat belt syndrome" was first described by Garret and Braunstein in 1962. The syndrome involves skin and abdominal wall ecchymosis (seat belt sign) intra-abdominal solid organ and visceral injuries, as well as Chance fractures (compression and/or wedging deformity of the anterior portion of the vertebral body with disruption or fracture of the posterior elements, generally at L1-L3). We present a case of a 12-year-old male involved in a high-speed motor vehicle collision wearing only a lap belt resulting in seat belt syndrome, with disruption of the abdominal wall, mesenteric avulsion with multiple intestinal perforations, abdominal aortic dissection, and an L2 Chance fracture with cord transection. Intraoperative decision making is outlined with this scenario of complex injuries, and the literature of seat belt syndrome associated with blunt aortic injuries and its management is reviewed.

  3. Self-Expandable Stent for Repairing Coarctation of the Left-Circumferential Aortic Arch with Right-sided Descending Aorta and Aberrant Right Subclavian Artery with Kommerell's Aneurysm.

    Science.gov (United States)

    Khajali, Zahra; Sanati, Hamid Reza; Pouraliakbar, Hamidreza; Mohebbi, Bahram; Aeinfar, Kamran; Zolfaghari, Reza

    2017-01-01

    Endovascular treatment offers a great advantage in the management of main arteries stenoses. However, simultaneous presence of a group of anomalies may complicate the situation. Here we present a case of 21-year-old man with aortic coarctation. Radiographic imaging and angiography demonstrated aortic coarctation of the left-circumferential aortic arch, right-sided descending aorta, and Kommerell's diverticulum at the origin of right subclavian artery. These anomalies have rarely been reported to concurrently exist in the same case and the treatment is challenging. Percutaneous treatment for repair of aortic coarctation was successfully performed with deployment of self-expanding nitinol stents. Follow-up demonstrated the correction of blood pressure and improvement of the symptoms. It appears that deployment of self-expandable nitinol stents present a viable option for the management of coarcted aorta in patients having all or some of these anomalies together.

  4. Unicuspid Aortic Stenosis in a Patient with Turner Syndrome: A Case Report.

    Science.gov (United States)

    Essandoh, Michael; Castellon-Larios, Karina; Zuleta-Alarcon, Alix; Portillo, Juan Guillermo; Crestanello, Juan A

    2014-01-01

    Congenital aortic valve anomalies are the cause of premature aortic stenosis in pediatric and younger adult populations. Despite being very rare, unicuspid aortic valves account for approximately 5% of isolated aortic valve replacements. Patients with aortic stenosis, present with the same symptomatology independent of leaflet morphology. However, the presence of bicuspid and unicuspid aortic stenosis is associated with a higher incidence of aortopathy, especially in Turner syndrome patients. Turner syndrome, an X monosomy, is associated with aortic valve anomalies, aortopathy, and hypertension. These risk factors lead to a higher incidence of aortic dissection in this population. Patients with Turner syndrome and aortic stenosis that present for aortic valve replacement should therefore undergo extensive aortic imaging prior to surgery. Transthoracic echocardiography is the diagnostic tool of choice for valvular pathology, yet it can misdiagnose unicuspid aortic valves as bicuspid valves due to certain similarities on imaging. Transesophageal echocardiography is a better tool for distinguishing between the two valvular abnormalities, although diagnostic errors can still occur. We present a case of a 50-year-old female with history of Turner syndrome and bicuspid aortic stenosis presenting for aortic valve replacement and ascending aorta replacement. Intraoperative transesophageal echocardiography revealed a stenotic unicommissural unicuspid aortic valve with an eccentric orifice, which was missed on preoperative imaging. This case highlights the importance of intraoperative transesophageal echocardiography in confirming preoperative findings, diagnosing further cardiac pathology, and ensuring adequate surgical repair.

  5. Unicuspid Aortic Stenosis in a Patient with Turner Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Michael eEssandoh

    2014-12-01

    Full Text Available Congenital aortic valve anomalies are the cause of premature aortic stenosis in pediatric and younger adult populations. Despite being very rare, unicuspid aortic valves account for approximately 5% of isolated aortic valve replacements. Patients with aortic stenosis, present with the same symptomatology independent of leaflet morphology. However, the presence of bicuspid and unicuspid aortic stenosis is associated with a higher incidence of aortopathy, especially in Turner syndrome patients. Turner syndrome, an X monosomy, is associated with aortic valve anomalies, aortopathy, and hypertension. These risk factors lead to a higher incidence of aortic dissection in this population. Patients with Turner syndrome and aortic stenosis that present for aortic valve replacement should therefore undergo extensive aortic imaging prior to surgery.Transthoracic echocardiography is the diagnostic tool of choice for valvular pathology, yet it can misdiagnose unicuspid aortic valves as bicuspid valves due to certain similarities on imaging. Transesophageal echocardiography is a better tool for distinguishing between the two valvular abnormalities, although diagnostic errors can still occur. We present a case of a 50-year-old female with history of Turner syndrome and bicuspid aortic stenosis presenting for aortic valve replacement and ascending aorta replacement. Intraoperative transesophageal echocardiography revealed a stenotic unicommissural unicuspid aortic valve with an eccentric orifice, which was missed on preoperative imaging. This case highlights the importance of intraoperative transesophageal echocardiography in confirming preoperative findings, diagnosing further cardiac pathology, and ensuring adequate surgical repair.

  6. An unusual case of multiple aortic abnormalities: total occlusion of aortic arch, left external iliac artery, and bicuspid aortic valve in a 21-year-old man.

    Science.gov (United States)

    Tanindi, Asli; Tavil, Yusuf; Mutluay, Ruya; Taktak, Hacer; Cengel, Atiye

    2007-03-01

    An unusual case of total occlusion of aorta just distal to the left subclavian artery, bicuspid aortic valve, and occluded left external iliac artery in a 21-year-old man who was admitted with headache and severe hypertension is presented. We wish to report this case because so far there have been none reported with such multiple aortic abnormalities, although several documented cases of isolated total occlusion of aorta exist. Our patient underwent a successful surgical correction, i.e., patch plasty to the coarcted segment and end to side - end to side aortal-aortal bypass with Dacron graft.

  7. Guyon's tunnel syndrome during pregnancy with concomitant anomalous arch of the ulnar nerve: a case report.

    Science.gov (United States)

    Janmohammadi, Nasser

    2014-01-01

    Numerous causes are reported for ulnar nerve compression at the wrist, known as Guyon's tunnel syndrome. In the present article, a patient with Guyon's tunnel syndrome during pregnancy concomitant with an anomaly of ulnar nerve is described. A 29-year-old Iranian woman presented with clinical features of Guyon's tunnel syndrome (pain and paresthesia in the fifth finger of the left hand and atrophy of the first dorsal interosseus muscle). Symptoms of the patient appeared during the third trimester of pregnancy. Electro diagnostic studies confirmed Guyon's tunnel syndrome. Surgical exploration revealed an anomalous arch of the ulnar nerve passing through the flexor carpi ulnaris (FCU) tendon. The anomalous arch of the ulnar nerve was released by resection of the segment of FCU tendon passing through the ulnar nerve arch. Therefore, in patients with Guyon's tunnel syndrome, the ulnar nerve anomaly should be kept in mind as a cause. Moreover, pregnancy may have a provocative effect on Guyon's tunnel syndrome similar to carpal tunnel syndrome (CTS).

  8. Guyon's tunnel syndrome during pregnancy with concomitant anomalous arch of the ulnar nerve: a case report.

    Directory of Open Access Journals (Sweden)

    Nasser Janmohammadi

    2014-07-01

    Full Text Available Numerous causes are reported for ulnar nerve compression at the wrist, known as Guyon's tunnel syndrome. In the present article, a patient with Guyon's tunnel syndrome during pregnancy concomitant with an anomaly of ulnar nerve is described. A 29-year-old Iranian woman presented with clinical features of Guyon's tunnel syndrome (pain and paresthesia in the fifth finger of the left hand and atrophy of the first dorsal interosseus muscle. Symptoms of the patient appeared during the third trimester of pregnancy. Electro diagnostic studies confirmed Guyon's tunnel syndrome. Surgical exploration revealed an anomalous arch of the ulnar nerve passing through the flexor carpi ulnaris (FCU tendon. The anomalous arch of the ulnar nerve was released by resection of the segment of FCU tendon passing through the ulnar nerve arch. Therefore, in patients with Guyon's tunnel syndrome, the ulnar nerve anomaly should be kept in mind as a cause. Moreover, pregnancy may have a provocative effect on Guyon's tunnel syndrome similar to carpal tunnel syndrome (CTS.

  9. Marfan Syndrome and Related Heritable Thoracic Aortic Aneurysms and Dissections.

    Science.gov (United States)

    De Backer, Julie; Renard, Marjolijn; Campens, Laurence; Mosquera, Laura Muino; De Paepe, Anne; Coucke, Paul; Callewaert, Bert; Kodolitsch, Yskert von

    2015-01-01

    In this overview we aim to address a number of recent insights and developments regarding clinical aspects, etiology, and treatment of Heritable Thoracic Aortic Disease (H-TAD). We will focus on monogenetic disorders related to aortic aneurysms. H-TADs are rare but they provide a unique basis for the study of underlying pathogenetic pathways in the complex disease process of aneurysm formation. The understanding of pathomechanisms may help us to identify medical treatment targets to improve prognosis. Among the monogenetic aneurysm disorders, Marfan syndrome is considered as a paradigm entity and many insights are derived from the study of clinical, genetic and animal models for Marfan syndrome. We will therefore first provide a detailed overview of the various aspects of Marfan syndrome after which we will give an overview of related H-TAD entities.

  10. A Rare Case of Pulmonary Atresia with Ventricular Septal Defect with a Right Sided Aortic Arch and a Calcified Pulmonary AVM Presenting in an Adult without Cyanosis

    Directory of Open Access Journals (Sweden)

    Devendra V. Kulkarni

    2014-01-01

    Full Text Available Pulmonary atresia with ventricular septal defect (PA-VSD with pulmonary arterial supply arising from the aorta representing large MAPCAs associated with a right sided aortic arch is an uncommon anomaly. Most of the patients succumb to severe respiratory compromise or congestive cardiac failure very early. We report the clinical details and imaging findings of a case of PA-VSD with a right sided aortic arch and a calcified pulmonary arteriovenous malformation (AVM in a 21-year-old postpartum female with no previous episodes of cyanosis who was diagnosed as having a cardiac anomaly on echocardiography when she presented with breathlessness during the 8th month of the pregnancy.

  11. 3D Computer Simulations of Pulsatile Human Blood Flows in Vessels and in the Aortic Arch: Investigation of Non-Newtonian Characteristics of Human Blood

    CERN Document Server

    Sultanov, Renat A; Engelbrekt, Brent; Blankenbecler, Richard

    2008-01-01

    Methods of Computational Fluid Dynamics are applied to simulate pulsatile blood flow in human vessels and in the aortic arch. The non-Newtonian behaviour of the human blood is investigated in simple vessels of actual size. A detailed time-dependent mathematical convergence test has been carried out. The realistic pulsatile flow is used in all simulations. Results of computer simulations of the blood flow in vessels of two different geometries are presented. For pressure, strain rate and velocity component distributions we found significant disagreements between our results obtained with realistic non-Newtonian treatment of human blood and widely used method in literature: a simple Newtonian approximation. A significant increase of the strain rate and, as a result, wall sear stress distribution, is found in the region of the aortic arch. We consider this result as theoretical evidence that supports existing clinical observations and those models not using non-Newtonian treatment underestimate the risk of disru...

  12. 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)

  13. High-risk pregnancy in a woman with Marfan syndrome, a bicuspid aortic valve, and a dilated aortic sinus

    DEFF Research Database (Denmark)

    Groth, Kristian Ambjørn; Greisen, Jacob Raben; Nielsen, Birgitte Bruun;

    2015-01-01

    A 29-year-old woman with Marfan syndrome, a bicuspid aortic valve, and a dilated aortic sinus (5.2 cm) presented herself in clinic 14 weeks pregnant. She was advised to discontinue the pregnancy due to risk of dissection; however, she decided to continue. She was treated with labetalol (300 mg...

  14. Hybrid repair of penetrating aortic ulcer associated with right aortic arch and aberrant left innominate artery arising from aneurysmal Kommerell's diverticulum with simultaneous repair of bilateral common iliac artery aneurysms.

    Science.gov (United States)

    Guo, Yuanyuan; Yang, Bin; Cai, Hongbo; Jin, Hui

    2014-02-01

    We present the first case of a hybrid endovascular approach to a penetrating aortic ulcer on the left descending aorta with a right aortic arch and aberrant left innominate artery arising from an aneurysmal Kommerell's diverticulum. The patient also had bilateral common iliac artery aneurysms. The three-step procedure consisted of a carotid-carotid bypass, followed by endovascular exclusion of the ulcer and the aneurysmal Kommerell's diverticulum, and then completion by covering the iliac aneurysms. The patient had no complications at 18 months after surgery. In such rare configurations, endovascular repair is a safe therapeutic option.

  15. Novel pharmacological strategies to prevent aortic complications in Marfan syndrome

    Institute of Scientific and Technical Information of China (English)

    Peter Matt; Friedrich Eckstein

    2011-01-01

    The Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the FBN1 gene.Recent molecular studies,most performed in mouse models,revealed that the MFS is more a developmental abnormality with broad and complex effects on the morphogenesis and function of multiple organ systems.FBN1 haploinsufficiency and dysregulated transforming growth factor-beta (TGF-β)signaling seem to be critical for clinical manifestations in MFS including aortic root dilatation.Aortic root aneurysm and aortic dissection represent the main causes of morbidity and mortality in MFS.Most importantly,TGF-β antagonism through angiotensin Ⅱ type 1 receptor blockers (ARBs),for example losartan,has been shown to prevent and possibly reverse aortic root dilatation in a mouse model of MFS.A first human study on a small pediatric cohort confirmed those promising results in reducing the aortic root growth over a follow-up period of 12 to 47 months.So,a large multicenter trial has been set up and results should be available soon.Other therapeutic strategies which might be combined with losartan include traditional β-blockade,doxycyclin and statins.Such management could offer the first potential for primary prevention of clinical manifestations in MFS.

  16. In vitro flow investigations in the aortic arch during cardiopulmonary bypass with stereo-PIV.

    Science.gov (United States)

    Büsen, Martin; Kaufmann, Tim A S; Neidlin, Michael; Steinseifer, Ulrich; Sonntag, Simon J

    2015-07-16

    The cardiopulmonary bypass is related to complications like stroke or hypoxia. The cannula jet is suspected to be one reason for these complications, due to the sandblast effect on the vessel wall. Several in silico and in vitro studies investigated the underlying mechanisms, but the applied experimental flow measurement techniques were not able to address the highly three-dimensional flow character with a satisfying resolution. In this work in vitro flow measurements in a cannulated and a non-cannulated aortic silicone model are presented. Stereo particle image velocimetry measurements in multiple planes were carried out. By assembling the data of the different measurement planes, quasi 3D velocity fields with a resolution of~1.5×1.5×2.5 mm(3) were obtained. The resulting velocity fields have been compared regarding magnitude, streamlines and vorticity. The presented method shows to be a suitable in vitro technique to measure and address the three-dimensional aortic CPB cannula flow with a high temporal and spatial resolution.

  17. Plasma Lactate Dehydrogenase Levels Predict Mortality in Acute Aortic Syndromes

    OpenAIRE

    Morello, Fulvio; Ravetti, Anna; Nazerian, Peiman; Liedl, Giovanni; Veglio, Maria Grazia; Battista, Stefania; Vanni, Simone; Pivetta, Emanuele; Montrucchio, Giuseppe; Mengozzi, Giulio; Rinaldi, Mauro; Moiraghi, Corrado; Lupia, Enrico

    2016-01-01

    Abstract In acute aortic syndromes (AAS), organ malperfusion represents a key event impacting both on diagnosis and outcome. Increased levels of plasma lactate dehydrogenase (LDH), a biomarker of malperfusion, have been reported in AAS, but the performance of LDH for the diagnosis of AAS and the relation of LDH with outcome in AAS have not been evaluated so far. This was a bi-centric prospective diagnostic accuracy study and a cohort outcome study. From 2008 to 2014, patients from 2 Emergency...

  18. Foot medial longitudinal-arch deformation during quiet standing and gait in subjects with medial tibial stress syndrome

    DEFF Research Database (Denmark)

    Bandholm, Thomas Quaade; Boysen, Lisbeth; Haugaard, Stine;

    2008-01-01

    The objective of this study was to investigate (1) if subjects with medial tibial stress syndrome demonstrate increased navicular drop and medial longitudinal-arch deformation during quiet standing and gait compared with healthy subjects, and (2) the relationship between medial longitudinal-arch ...

  19. Aortic and Cardiac Structure and Function Using High-Resolution Echocardiography and Optical Coherence Tomography in a Mouse Model of Marfan Syndrome

    Science.gov (United States)

    Lee, Ling; Cui, Jason Z.; Cua, Michelle; Esfandiarei, Mitra; Sheng, Xiaoye; Chui, Winsey Audrey; Xu, Michael Haoying; Sarunic, Marinko V.; Beg, Mirza Faisal; van Breemen, Cornelius; Sandor, George G. S.

    2016-01-01

    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; pMarfan 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. PMID:27824871

  20. 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.

  1. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial

    NARCIS (Netherlands)

    Groenink, M.; Hartog, A.W. den; Franken, R.; Radonic, T.; Waard, V. de; Timmermans, J.; Scholte, A.J.; Berg, M.P van den; Spijkerboer, A.M.; Marquering, H.A.; Zwinderman, A.H.; Mulder, B.J.

    2013-01-01

    AIM: Patients with Marfan syndrome have an increased risk of life-threatening aortic complications, mostly preceded by aortic dilatation. Treatment with losartan, an angiotensin-II receptor-1 blocker, may reduce aortic dilatation rate in Marfan patients. METHODS AND RESULTS: In this multicentre, ope

  2. Losartan reduces aortic dilatation rate in adults with Marfan syndrome : a randomized controlled trial

    NARCIS (Netherlands)

    Groenink, Maarten; den Hartog, Alexander W.; Franken, Romy; Radonic, Teodora; de Waard, Vivian; Timmermans, Janneke; Scholte, Arthur J.; van den Berg, Maarten P.; Spijkerboer, Anje M.; Marquering, Henk A.; Zwinderman, Aeilko H.; Mulder, Barbara J. M.

    2013-01-01

    Aim Patients with Marfan syndrome have an increased risk of life-threatening aortic complications, mostly preceded by aortic dilatation. Treatment with losartan, an angiotensin-II receptor-1 blocker, may reduce aortic dilatation rate in Marfan patients. Methods and results In this multicentre, open-

  3. Continuous measurement of aortic dimensions in Turner syndrome

    DEFF Research Database (Denmark)

    Subramaniam, Dhananjay Radhakrishnan; Stoddard, William A; Mortensen, Kristian H

    2017-01-01

    method was then compared with corresponding manual measurements at 7 discrete locations for each visit and for changes between visits. RESULTS: Manual measures at the seven positions and the corresponding continuous measurements of maximum diameter for all visits considered, correlated highly (R-value...... methodology is presented that enables rapid and precise three-dimensional measurement of thoracic aortic geometry, which can serve as an improved tool to define disease severity and monitor disease progression. TRIAL REGISTRATION: ClinicalTrials.gov Identifier - NCT01678274 . Registered - 08.30.2012.......BACKGROUND: Severity of thoracic aortic disease in Turner syndrome (TS) patients is currently described through measures of aorta size and geometry at discrete locations. The objective of this study is to develop an improved measurement tool that quantifies changes in size and geometry over time...

  4. Clinical and biochemical outcomes for additive mesenteric and lower body perfusion during hypothermic circulatory arrest for complex total aortic arch replacement surgery.

    Science.gov (United States)

    Fernandes, P; Cleland, A; Adams, C; Chu, M W A

    2012-11-01

    Surgical repair of transverse aortic arch aneurysms frequently employ hypothermia and antegrade cerebral perfusion as protective strategies during circulatory arrest. However, prolonged mesenteric and lower limb ischemia can lead to significant lactic acidosis and end organ dysfunction, which remains a significant cause of post-operative morbidity and mortality. We report our experience with additive warm mesenteric and lower body perfusion (1-3 L/min, 30°C) in addition to continuous cerebral and myocardial perfusion in 5 patients who underwent total aortic arch replacement with trifurcated head vessel re-implantation and distal elephant trunk reconstruction. Concomitant surgical procedures included re-operations (2), aortic root operations (2), coronary artery bypass (2) and descending thoracic aortic replacement (1). Serum lactate levels demonstrated a rapid decline from a peak 9.9 ± 2.6 post circulatory arrest to 3.4 ± 2.0 in the intensive care unit (ICU). The lowest serum bicarbonate levels were 19.3 ± 3.5 mmol/L, intra-operatively, which normalized to 28.4 ± 2.4 mmol/L on return to the ICU. The lowest pH levels were 7.25 ± 0.10, corrected to 7.43 ± 0.04 on return to the ICU. Mean cardiopulmonary bypass and aortic cross-clamp times were 361 ± 104 and 253 ± 85 minutes, respectively. Mean cerebral and lower body circulatory arrest times were 0 (0) and 50 ± 35 minutes, respectively. The mean time required for systemic rewarming was 95 ± 66 minutes. There were no in-hospital mortalities and no patient experienced any neurological, mesenteric, renal or lower limb ischemic complications. Two patients required mechanical ventilation >24 hours, and one patient returned for reoperation for bleeding. Median intensive care unit and total hospital lengths of stay were 5 and 16 days, respectively. Our results suggest early serum lactate clearance, normalization of acidosis, and metabolic recovery when utilizing a simultaneous cerebral perfusion and warm body

  5. One-stage Surgical Correction of Aortic Coarctation Complicated With Aortic Arch Hypoplasia by Autologous Pulmonary Artery Patch%自体肺动脉补片一期矫治主动脉缩窄伴主动脉弓发育不良

    Institute of Scientific and Technical Information of China (English)

    王显悦; 毕生辉; 童光; 董文鹏; 王晓武; 梁爱琼; 徐宇; 张卫达

    2014-01-01

      结论:自体肺动脉补片一期矫治主动脉缩窄伴弓发育不良,病变解除良好,并发症少,手术后早中期效果理想。%Objective: To observe the outcomes of one-stage surgical correction of aortic coarctation (COA) complicated with aortic arch hypoplasia by autologous pulmonary artery patch. Methods: A total of 22 COA with aortic arch hypolasia children treated in our hospital from 2009-05 to 2013-05 were summarized. All patients were clearly diagnosed by CTA. All patients received the one-stage surgical correction of aortic coarctation complicated with aortic arch hypoplasia by autologous pulmonary artery patch. The selective low-lfow cerebral perfusion was used during aortic arch procedure and the concomitant cardiac anomalies were corrected during the same surgery. Results: No peri-operative death. There were 6 patients with the upper arm BP higher than lower arm immediately after the operation and the pressure gradient Conclusion: COA with aortic arch hypoplasia could be treated with one-stage surgical correction using autologous pulmonary artery patch, which had good early and mid term outcomes.

  6. 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 referri

  7. Aortic anomalies in an adolescent with the Williams' elfin facies syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Williams, R.L.; Azouz, E.M.

    1984-02-01

    An adolescent with classical Williams' syndrome who presented with hypertensive encephalopathy is described. He had the unusual combination of supravalvular aortic stenosis, long segment coarctation of the aorta, aortic hypoplasia and a high bifurcation of the abdominal aorta. Surgical resection of the coarctation was required; however, the patient has remained moderately hypertensive. The aortic anomalies in this syndrome are reviewed and their frequency and importance examined.

  8. Prenatal ultrasonic diagnosis of fetal interrupted aortic arch%胎儿主动脉弓离断产前超声诊断分析

    Institute of Scientific and Technical Information of China (English)

    董莹; 王玲; 赵晟; 郭宁; 肖蕾

    2014-01-01

    目的:探讨产前超声对胎儿主动脉弓离断(IAA)的诊断价值,分析漏、误诊原因,提高产前诊断率。方法回顾性分析16例胎儿主动脉弓异常超声图像特征,与正常胎儿超声图像、解剖结果进行对比研究,并分析胎儿IAA与染色体异常的相关性。结果16例产前诊断IAA病例,经解剖确诊15例,超声诊断正确率为94%(15/16),1例为主动脉弓严重缩窄(CoA),1例分型错误,3例产前超声不能分型病例获得明确分型,超声分型符合率为73%(11/15)。确诊的15例全部合并室间隔缺损,其中5例合并完全性心内膜垫缺损,合并心脏外畸形者9例。7例进行了染色体核型检查,3例18‐三体,1例13‐三体,3例正常,18‐三体发生率为43%(3/7)。结论产前超声对胎儿IAA的诊断准确率高,但在明确分型方面有待提高。胎儿IAA与严重CoA的鉴别存在困难。18‐三体在IAA胎儿染色体异常中发生率较高。%Objective To evaluate the value of prenatal ultrasound in the interrupted aortic arch (IAA), and analyze the reasons of misdiagnosis and improve diagnostic ratio of this kind of defects. Methods Ultrasonic characteristics were analyzed in 16 fetuses with aortic arch anomalies, which were compared with ultrasound image and autopsy results of the normal fetus. The relativity of fetal IAA and abnormal chromosome was also analyzed. Results Among the 16 cases, 15 cases were confirmed by anatomy and the accuracy of ultrasound diagnosis was 94% (15/16), of which one case was serious constriction of aortic arch, one case was error type, three cases were obtained explicit type due to unsatisfactory results of prenatal ultrasound. The coincidence rate of ultrasonic type was 73% (11/15). All of the corrected diagnosed cases were detected with ventricular septal, of which 5 cases were detected with complete endocardial cushion defect, 9 cases were found extra cardiac

  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. Aorta-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch: a clinical analysis of 5 cases%主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断(附5例报告)

    Institute of Scientific and Technical Information of China (English)

    张刚成; 韩霞; 李艳萍; 陶凉

    2011-01-01

    pulmonary artery with interruption of the aortic arch ( with intact ventricular septum) are different from the classic triad of inter ruption of the aortic arch, consisted of the interruption of the aortic arch, patent ductts arteriosus and ventricular septal defect.It should be considered as a special kind of cardiovascular malformation syndrome. Echocardiography can be used for the preliminary diagnosis of aorta-pulmonary septal defect and aortic origin of right pulmonary artery with interruption of the aortic arch. The final diagnosis and decision for surgery should be based on the combined information from cardiac catheterization,cardiovascular angiography and spiral CT examination. Pulmonary artery pressure and resistance are the most important factors,because they have great effects on the duration of surgery and the outcome. A radical operation is necessary for all cases, but the mortality rate is high. The prognosis would be great if only the patient can survive successfully during the peri-operative period, a period with a poor cardiac function, potential lung infection and the pulmonary artery hypertension.%目的 探讨主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的发病情况,总结诊断和治疗经验.方法 回顾性分析武汉亚洲心脏病医院2005至2009年5例病儿,均经超声心动图、螺旋CT检查,3例10岁以上病儿行心导管检查及心血管造影,确诊为A型主动脉弓离断、Ⅱ型主-肺动脉缺损、右肺动脉起源于升主动脉,均合并粗大动脉导管未闭、无室间隔缺损等心内畸形.年龄1~18岁,其中男4例,女1例.结果 1例18岁病人,应用3个月前列环素(万他维)降肺动脉压力治疗,现心功能Ⅱ级;1例14岁病儿放弃治疗,失访.余3例行一期根治手术,其中1岁病婴并发严重肺部感染,术后第11天死亡;2岁病婴术后仍有重度肺动脉高压,经强心、利尿及降肺动脉压治疗,随访18个月

  11. 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

  12. 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

    2008-01-01

    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

  13. Chronic atrial fibrillation in presence of aortic stenosis in a patient with polysplenia syndrome.

    Science.gov (United States)

    Bronte, E; Trovato, Rl; Di Miceli, R; Sucato, V; Candela, P; Brancatelli, G; Novo, S

    2013-01-01

    We report a rare case of "situs viscerum ambiguous" with polysplenia syndrome, in a 69 year old female patient with aortic stenosis and chronic atrial fibrillation. The presenting symptom was dyspnoea on moderate exertion and an ECG showed supra ventricular arrhythmia. Patients trans-thoracic echocardiogram revealed a dilated left atrium, reduced ejection fraction, mild tricuspid regurgitation, moderate-severe pulmonary hypertension and severe aortic stenosis. The patient was successfully treated with a replacement of her aortic valve and ascending aorta.

  14. 主动脉弓滑动成形术治疗婴幼儿主动脉缩窄并主动脉弓发育不良的临床研究%Clinical study of aortic arch sliding aortoplasty in surgical repair of infants with coarctation of the aorta and hypoplastic aortic arch

    Institute of Scientific and Technical Information of China (English)

    熊红燕; 朱海龙; 孙国成; 魏东明; 俞世强

    2016-01-01

    目的:总结运用主动脉弓滑动成形术治疗婴幼儿主动脉缩窄合并主动脉弓发育不良的临床经验。方法回顾性分析2013年1月至2015年5月间采用主动脉弓滑动成形术治疗主动脉缩窄合并主动脉弓发育不良患儿8例的临床资料。患儿年龄(7.0±5.4)个月(2~21个月),体重(6.6±1.7)kg (4~10 kg),男性6例,女性2例。均经胸骨正中切口在深低温体外循环和选择性脑灌注下,一期完成手术。根据患儿主动脉病理解剖的不同,主动脉弓滑动成形方法略有不同,以达到最好的解剖矫治效果。结果8例患儿均成功完成手术。全组无死亡,未出现肾功能障碍及神经系统功能并发症。术后定期随访(16.0±5.6)个月(6~20个月),末次随访患者下肢收缩压均高于上肢收缩压(8.9±3.2) mmHg (0~15 mmHg)(1 mmHg=0.133 kPa),未发现再狭窄。结论主动脉弓滑动成形术治疗婴幼儿主动脉缩窄合并主动脉弓发育不良具有良好的近中期效果。%Objective To summarize the clinical experiences of aortic arch sliding aortoplasty in surgical repair of infants with coarctation of the aorta and hypoplastic aortic arch.MethodsEight patients with coarctation of the aorta and hypoplastic aortic arch who underwent surgical repair by aortic arch sliding aortoplasty from January 2013 to May 2015 were retrospectively analyzed. The patients' age was (7±5.4) months (2~21 months) and weight was (6.6±1.7) kg (4~10 kg). Six of them were male and two of them were female. All the operations were performed through median sternotomy under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion. According to the pathological anatomy of aortic arch, the techniques were different to reach optimal anatomical repair.ResultsAll patients were repaired successfully. There was no mortality or major complication such as renal dysfunction or nervous system complications

  15. Postural orthostatic tachycardia syndrome after surgical correction of an aortic coarctation: a case report

    Directory of Open Access Journals (Sweden)

    Fernex Lucie

    2012-08-01

    Full Text Available Abstract Introduction We report a case of postural tachycardia syndrome occurring after the surgical correction of an aortic coarctation, and coexisting with upper airway resistance syndrome. Case presentation A 29-year-old Caucasian man complained of extreme fatigue, daytime sleepiness, shortness of breath on exertion, light-headedness and general weakness on standing. These symptoms began shortly after the surgical correction of an aortic coarctation and became progressively more debilitating, impairing any daily activity. An extensive work-up revealed postural tachycardia syndrome and a coexisting sleep-related breathing disorder, characterized as upper airway resistance syndrome. Conclusion This is the first reported case describing the occurrence of postural tachycardia syndrome after the surgical correction of an aortic coarctation. This case also provides evidence for the suggestion that this syndrome may coexist with upper airway resistance syndrome, although the exact nature of their relationship must still be better established.

  16. Thoracic Aortic Disease in Two Patients with Juvenile Polyposis Syndrome and SMAD4 mutations

    Science.gov (United States)

    Teekakirikul, Polakit; Milewicz, Dianna M.; Miller, David T.; Lacro, Ronald V.; Regalado, Ellen S.; Rosales, Ana Maria; Ryan, Daniel P.; Toler, Tomi L.; Lin, Angela E.

    2012-01-01

    Dilation or aneurysm of the ascending aorta can progress to acute aortic dissection (Thoracic Aortic Aneurysms and Aortic Dissections, TAAD). Mutations in genes encoding TGF-β related proteins (TGFBR1, TGFBR2, FBN1, and SMAD3) cause syndromic and inherited TAAD. SMAD4 mutations are associated with juvenile polyposis (JPS) and a combined JPS-hereditary hemorrhagic telangiectasia (HHT) known as JPS-HHT. A family with JPS-HHT was reported to have aortic root dilation and mitral valve abnormalities. We report on two patients with JPS-HHT with SMAD4 mutations associated with thoracic aortic disease. The first patient, an 11-year-old boy without Marfan syndrome features, had JPS and an apparently de novo SMAD4 mutation (c.1340_1367dup28). Echocardiography showed mild dilation of the aortic annulus and aortic root, and mild dilation of the sinotubular junction and ascending aorta. Computed tomography confirmed aortic dilation and showed small pulmonary arteriovenous malformations (PAVM). The second patient, a 34-year-old woman with colonic polyposis, HHT, and Marfan syndrome, had a SMAD4 mutation (c.1245_1248delCAGA). Echocardiography showed mild aortic root dilation. She also had PAVM and hepatic focal nodular hyperplasia. Her family history was significant for polyposis, HHT, thoracic aortic aneurysm, and dissection and skeletal features of Marfan syndrome in her father. These two cases confirm the association of thoracic aortic disease with JPS-HHT resulting from SMAD4 mutations. We propose that the thoracic aorta should be screened in patients with SMAD4 mutations to prevent untimely death from dissection. This report also confirms that SMAD4 mutations predispose to TAAD. PMID:23239472

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

    NARCIS (Netherlands)

    Doyle, A.J.; Doyle, J.J.; Bessling, S.L.; Maragh, S.; Lindsay, M.E.; Schepers, D.; Gillis, E.; Mortier, G.; Homfray, T.; Sauls, K.; Norris, R.A.; Huso, N.D.; Leahy, D.; Mohr, D.W.; Caulfield, M.J.; Scott, A.F.; Destree, A.; Hennekam, R.C.; Arn, P.H.; Curry, C.J.; Laer, L. van; McCallion, A.S.; Loeys, B.L.; Dietz, H.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). However, the location and character of many of the causal mutations in LDS intuitively imply

  18. Management of severe asymmetric pectus excavatum complicating aortic repair in a patient with Marfan's syndrome.

    Science.gov (United States)

    Yeung, Jonathan C; Marcuzzi, Danny; Peterson, Mark D; Ko, Michael A

    2016-05-01

    We describe the case of a 28-year old man with Marfan's syndrome and severe pectus excavatum who required an aortic root replacement for an ascending aortic aneurysm. There was a near-vertical angulation of the sternum that presented challenges with opening and exposure of the heart during aortic surgery. Furthermore, removal of the sternal retractor after aortic repair resulted in sudden loss of cardiac output. A Ravitch procedure was then performed to successfully close the chest without further cardiovascular compromise. We propose that patients with a severe pectus excavatum and mediastinal displacement seen on preoperative CT scanning should be considered for simultaneous, elective repair.

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

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun; Choi, Dong Hyun; Lee, Young Min; Kim, Bo Bae; Ki, Young Jae; Kim, Jin Hwa; Chung, Joong Wha; Koh, Young Youp [Chosun University School of Medicine, Gwangju (Korea, Republic of); Kang, Joon Tae; Chae, Seung Seok [Dept. of nternal Medicine, Mokpo Jung-Ang General Hospital, Mokpo (Korea, Republic of)

    2014-10-15

    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.

  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. MDCT and 3D evaluation of type 2 hypoplastic pulmonary artery sling associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis.

    Science.gov (United States)

    Lee, Edward Y

    2007-11-01

    The early diagnosis and complete anatomic evaluation of pulmonary artery sling, a congenital vascular anomaly in which left pulmonary artery arises from the right pulmonary artery, is paramount for proper patient management, because patients with this disorder frequently have other congenital anomalies resulting in high morbidity and mortality. Until recently, pulmonary artery sling in the neonate has been established with standard radiologic imaging studies such as plain radiographs, barium swallow studies, fluoroscopy-guided airway studies, and echocardiograms. However, with the development and widespread availability of multidetector computed tomography, pulmonary artery sling is increasingly evaluated with this newer technology. This case report presents a rare incidence of type 2 hypoplastic pulmonary artery sling in a neonate associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis. Multidetector computed tomography combined with 3-dimensional evaluation was particularly helpful in making a correct diagnosis of the complicated anatomic anomalies found in this case.

  2. Aortic dilatation in Turner syndrome: the role of MRI in early recognition

    Energy Technology Data Exchange (ETDEWEB)

    Chalard, Francois; Ferey, Solene; Kalifa, Gabriel [Saint Vincent de Paul Hospital, Department of Paediatric Radiology, Paris Cedex 14 (France); Teinturier, Cecile [Saint Vincent de Paul Hospital, Department of Paediatric Endocrinology, Paris (France)

    2005-03-01

    Aortic dilatation and dissection are rare but important complications of Turner syndrome that increase the risk of sudden death in young patients. To assess the value of aortic MRI in patients with Turner syndrome; in particular to demonstrate early aortic dilatation. A total of 21 patients with Turner syndrome underwent MRI of the thoracic aorta with measurement of vessel diameter at four levels. Measurements were normal for age in 15 cases, two patients presented with values at the upper limit of normal and four had obvious dilatation of the ascending aorta. All were symptom free. MRI allows the non-invasive demonstration of early aortic dilatation, which may lead to earlier surgery in asymptomatic individuals. (orig.)

  3. Tratamento cirúrgico da compressão do esôfago por anomalia do arco aórtico: relato de caso Surgical treatment of esophagus compression by aortic arch anomaly: case report

    Directory of Open Access Journals (Sweden)

    Ivo Richter

    1991-08-01

    Full Text Available Paciente adulta, portadora de anomalia vascular congênita do arco aórtico com formação de anel envolvendo traqueia e esôfago, com compressão deste e conseqüente disfagia progressiva. A abordagem cirúrgica foi realizada por toracotomia lateral direita, com ressecção do divertrículo de Kommerell, secção do ligamento arterioso e ligadura simples da artéria subclávia, com regressão total dos sintomas. Não foi observada síndrome de roubo da subclávia ou isquemia do membro superior esquerdo. Os autores discutem o tipo de abordagem e cirurgia para tratamento desta rara anomalia.An adult patient presenting a congenital vascular anomaly of the aortic arch and ring formation, surrounding trachea and esophagus, by compression of the latest was complaining of progressive dysphagia. A surgical approach was performed by right lateral thoracotomy with ressection of the Kommerell diverticulum, section of the arterious ligament and single ligation of the subclavian artery which was followed by total regression of the symptoms. No subclavian steal syndrome or ischemia was observed on the left upper limb. The authors discuss the manner of approach and surgical treatment of this rare anomaly.

  4. Surgical repair of left-sided cervical aortic arch aneurysm%左颈位主动脉弓动脉瘤的外科治疗

    Institute of Scientific and Technical Information of China (English)

    郑铁; 朱俊明; 孙立忠; 钟永亮; 齐瑞东; 程力剑; 葛翼鹏; 陈雷; 刘巍; 里程楠; 邢晓燕

    2016-01-01

    目的:总结颈位主动脉弓(cervical aortic arch,CAA)动脉瘤的外科治疗方法及术后早中期效果。方法2010年1月至2014年12月,收治22例CAA动脉瘤患者,均为左位CAA。其中男6例,女16例,年龄(34.09±13.14)岁。术前合并主动脉假性狭窄9例,高血压3例,主动脉瓣反流、Stanford B型主动脉夹层、大脑中动脉瘤各1例。所有患者均采用人工血管替换术行主动脉弓重建。4例(4/22,18.18%)经胸骨正中切口,中度低温停循环、选择性顺行脑灌注下手术,其中同期行主动脉瓣置换术1例;18例(18/22,81.82%)经左后外侧第4肋间切口手术,其中10例体外循环辅助下实施手术,8例直接阻断下手术。结果全组平均呼吸机辅助(13.05±4.73) h,ICU停留(19.14±8.08) h。术后二次开胸止血1例,切口延迟愈合1例,一过性肝功能不全1例。无院内死亡。术后19例随访平均34.73个月,失访3例。随访期间无死亡。结论 CAA动脉瘤患者应积极手术治疗。根据CAA动脉瘤的位置、类型以及合并的其他近端心血管疾病,选择个体化的手术切口、辅助技术及手术方法,一期行主动脉弓重建手术治疗CAA动脉瘤可获得满意的临床效果。%Objective To summarize our experience of surgical repair for cervical aortic arch(CAA) aneurysm and eval-uate early and midterm results of these patients.Methods From January 2010 to December 2014, 22 patients with left-sided CAA aneurysm admitted in our center.There were 6 male and 16 female patients with a mean age of(34.09 ±13.14) years. Comorbidities included pseudocoarctation in 9 patients, hypertension in 4 patients, and aortic valve insufficiency, Stanford type B aortic dissection and middle cerebral artery aneurysm each had 1 patient.All of the patients underwent surgical aortic arch re-construction using artificial graft replacement.Among them, 4(4/22, 18.18%) were

  5. Singleton Merten Syndrome: A Rare Cause of Early Onset Aortic Stenosis

    Science.gov (United States)

    Mungee, Sudhir

    2017-01-01

    Singleton Merten syndrome (SMS) is a rare autosomal dominant genetic disorder with variable expression. Its characteristic features include abnormal aortic calcification, abnormal ossification of extremities, and dental anomalies. We present a young man with dyspnea who was noted to have aortic stenosis in the background of glaucoma, psoriasis, dental anomalies, hand and foot deformities, Achilles tendinitis, osteopenia, and nephrolithiasis. The conglomeration of features led to the diagnosis of SMS. His mother had a very similar phenotype. PMID:28321341

  6. Singleton Merten Syndrome: A Rare Cause of Early Onset Aortic Stenosis

    Directory of Open Access Journals (Sweden)

    Harshavardhan Ghadiam

    2017-01-01

    Full Text Available Singleton Merten syndrome (SMS is a rare autosomal dominant genetic disorder with variable expression. Its characteristic features include abnormal aortic calcification, abnormal ossification of extremities, and dental anomalies. We present a young man with dyspnea who was noted to have aortic stenosis in the background of glaucoma, psoriasis, dental anomalies, hand and foot deformities, Achilles tendinitis, osteopenia, and nephrolithiasis. The conglomeration of features led to the diagnosis of SMS. His mother had a very similar phenotype.

  7. Plasma Lactate Dehydrogenase Levels Predict Mortality in Acute Aortic Syndromes

    Science.gov (United States)

    Morello, Fulvio; Ravetti, Anna; Nazerian, Peiman; Liedl, Giovanni; Veglio, Maria Grazia; Battista, Stefania; Vanni, Simone; Pivetta, Emanuele; Montrucchio, Giuseppe; Mengozzi, Giulio; Rinaldi, Mauro; Moiraghi, Corrado; Lupia, Enrico

    2016-01-01

    Abstract In acute aortic syndromes (AAS), organ malperfusion represents a key event impacting both on diagnosis and outcome. Increased levels of plasma lactate dehydrogenase (LDH), a biomarker of malperfusion, have been reported in AAS, but the performance of LDH for the diagnosis of AAS and the relation of LDH with outcome in AAS have not been evaluated so far. This was a bi-centric prospective diagnostic accuracy study and a cohort outcome study. From 2008 to 2014, patients from 2 Emergency Departments suspected of having AAS underwent LDH assay at presentation. A final diagnosis was obtained by aortic imaging. Patients diagnosed with AAS were followed-up for in-hospital mortality. One thousand five hundred seventy-eight consecutive patients were clinically eligible, and 999 patients were included in the study. The final diagnosis was AAS in 201 (20.1%) patients. Median LDH was 424 U/L (interquartile range [IQR] 367–557) in patients with AAS and 383 U/L (IQR 331–460) in patients with alternative diagnoses (P < 0.001). Using a cutoff of 450 U/L, the sensitivity of LDH for AAS was 44% (95% confidence interval [CI] 37–51) and the specificity was 73% (95% CI 69–76). Overall in-hospital mortality for AAS was 23.8%. Mortality was 32.6% in patients with LDH ≥ 450 U/L and 16.8% in patients with LDH < 450 U/L (P = 0.006). Following stratification according to LDH quartiles, in-hospital mortality was 12% in the first (lowest) quartile, 18.4% in the second quartile, 23.5% in the third quartile, and 38% in the fourth (highest) quartile (P = 0.01). LDH ≥ 450 U/L was further identified as an independent predictor of death in AAS both in univariate and in stepwise logistic regression analyses (odds ratio 2.28, 95% CI 1.11–4.66; P = 0.025), in addition to well-established risk markers such as advanced age and hypotension. Subgroup analysis showed excess mortality in association with LDH ≥ 450 U/L in elderly, hemodynamically stable

  8. 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.

  9. 主动脉弓手术后并发谵妄的临床分析%Clinical analysis of postoperative delirium following aortic arch operation

    Institute of Scientific and Technical Information of China (English)

    袁辉; 祁明; 黄维勤

    2013-01-01

    目的:分析主动脉弓手术后谵妄的发生率和危险因素.方法:回顾分析143例主动脉弓手术后谵妄发生率,按有无谵妄症状分成2组,对其谵妄的可能危险因素做统计学分析.结果:3例患者术后早期因心肺功能衰竭死亡外,谵妄的发生率为29.3%(41/140例).术后谵妄的危险因素包括:术前高血压病、脑血管疾病、糖尿病、术中低脑氧饱和度、体外循环(CPB)> 200 min、术中最低收缩压、最低血压持续时间、术后发热、输血量>3 000 ml.结论:主动脉弓术后谵妄发生率为29.3%,术中最低收缩压、最低血压持续时间及CPB> 200 min、术中脑氧饱和度<40%是谵妄发生的独立危险因素.%AIM:To observe the incidence,risk factors and prevention methods of delirium after aortic arch operation.METHODS:The incidence of delirium in 143 patients who received aortic arch surgery was retrospectively analyzed.Patients were divided into two groups according to occurrence or no occurrence of postoperative delirium.Possible delirium-causing risk factors were analyzed.RESULTS:Except for early postoperative death due to cardiac and pulmonary failure in three patients,the incidence of delirium was 29.3% (4i/140).Risk factors for postoperative delirium included 1) preoperative diseases such as hypertension,cerebrovascular diseases and diabetes mellitus; 2) lower cerebral oxygen saturation,cardiopulmonary bypass (CPB) > 200 min,minimum systolic blood pressure and duration of minimum blood pressure during operation,and 3) postoperative fever and blood transfusion volume > 3 000 ml.CONCLUSION:Minimum systolic blood pressure and duration of minimum blood pressure,CPB > 200min and cerebral oxygen saturation < 40% during operation are independent risk factors for the occurence of delirium.

  10. Late pseudocoarctation syndrome after stent-graft implantation for traumatic aortic rupture.

    Science.gov (United States)

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

    2013-06-01

    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.

  11. 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.

  12. Aortic and Pulmonary Root Aneurysms in a Child With Loeys-Dietz Syndrome.

    Science.gov (United States)

    Rizzo, Stefania; Stellin, Giovanni; Milanesi, Ornella; Padalino, Massimo; Vricella, Luca A; Thiene, Gaetano; Cameron, Duke E; Basso, Cristina; Vida, Vladimiro L

    2016-03-01

    We report the case of an 11-year-old boy with Loeys-Dietz syndrome, with both aortic and pulmonary aneurysms requiring cardiac operation because of progressive valve incompetence resulting from loss of coaptation of the cusps. Arterial medial changes, consisting of disarray of elastic fibers and increased collagen deposition, were observed in surgical specimens from both the aorta and the pulmonary artery of our patient, and the strong pSmad2 nuclear staining of smooth muscle cells of both aortic and pulmonary tunica media are the best evidence of transforming growth factor-β pathway activation in Loeys-Dietz syndrome.

  13. Aortic stent graft infection in a patient with Marfan syndrome

    Institute of Scientific and Technical Information of China (English)

    FU Wei-guo; SHI De-bing; WANG Yu-qi; GUO Da-qiao; CHEN Bin; SHI Zhen-yu

    2007-01-01

    @@ Endovascular repair of ruptured abdominal aortic aneurysm (AAA) has been adopted clinically.1, 2 It is especially suitable for the patients who are at high surgical risk or fulfil the technical requirements for endovascular therapy.

  14. Pitfalls in Suspected Acute Aortic Syndrome: Impact of Appropriate and If Required Repeated Imaging

    Directory of Open Access Journals (Sweden)

    C. Meier

    2015-01-01

    Full Text Available The incidence of acute aortic syndrome is low, but the spontaneous course is often life-threatening. Adequate ECG-gated imaging is fundamental within the diagnostic workup. We here report a case of a 53-year-old man presenting with atypical chest pain, slight increase of D dimers at admission, and extended diameter of the ascending aorta accompanied by mild aortic regurgitation. Interpretation of an initial contrast-enhanced computed tomography was false negative due to inadequate gating and motion artifacts, thereby judging a tiny contrast signal in the left anterior quadrant of the ascending aorta as a pseudointimal flap. By hazard, cardiac magnetic resonance imaging demonstrated an ulcer-like lesion superior to the aortic root, leading to aortic surgery at the last moment. As sensitivity of imaging is not 100%, this example underlines that second imaging studies might be necessary if the first imaging is negative, but the clinical suspicion still remains high.

  15. Aortic dimensions in girls and young women with turner syndrome: a magnetic resonance imaging study

    DEFF Research Database (Denmark)

    Mortensen, Kristian H; Skouby, Sven O; Leffers, Anne-Mette

    2010-01-01

    This study aimed to determine the dimensions of the thoracic aorta and the predictors of aortic dimensions in girls and young women with Turner syndrome (TS). A cross-sectional study was performed at a secondary care center. The study compared 41 TS patients with 50 healthy age-matched control...... 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...... 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 aorta correlated with a history of aortic coarctation (R = 0.35-0.52; p

  16. Exploring novel treatments to prevent aortic aneurysm growth in Marfan syndrome

    NARCIS (Netherlands)

    Hibender, S.

    2016-01-01

    Widening of the aorta (aneurysm) may occur during ageing or in patients with genetic predisposition, such as in Marfan Syndrome (MFS). Aneurysm growth eventually leads to aortic rupture and death. Currently no pharmacological treatment is present to combat aneurysm growth. In this thesis, treatment

  17. 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 in

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

    OpenAIRE

    S. Gupta; B. Goswami; 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.

  19. Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child

    OpenAIRE

    2013-01-01

    Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated...

  20. [Post-cesarean acute aortic dissection in a Marfan syndrome patient].

    Science.gov (United States)

    Onofriescu, M; Gavriluţ, Maria; Tinică, G; Diaconescu, V; Holicov, Monica; Radu, E; Aldea, Marie-Jeanne

    2007-01-01

    Marfan syndrome is an uncommon condition in pregnancy. We present the case of 37 years old gravida 1, para 1 with Marfan syndrome. She delivered at term by cesarean section, a healthy male infant weighing 3500 grams with Apgar's of 9. During the postoperative period she developed aortic dissection and was referred to the Cardiovascular Surgery Department. We described such a case and the difficult decisions that we faced.

  1. Advances of surgical and perfusion techniques for aortic arch hypoplasia%小儿主动脉弓发育不良的手术及灌注技术进展

    Institute of Scientific and Technical Information of China (English)

    毛乐; 祝忠群

    2016-01-01

    Currently the anatomic criteria for defining aortic arch hypoplasia have remained controversial.The major circumstances are proximal transverse aortic arch of <60% of ascending aortic diameter,a distal transverse aortic arch of <50% of ascending aortic diameter,an isthmus < 40%,a diameter of transverse arch < 50% of distal ascending aortic diameter or a diameter of transverse arch <50% of distal descending aortic diameter.One common rule-of-thumb is a diameter of transverse arch <0.1 mm for each 1 kg body weight,z-score:the diameter of each segment < two standard deviations below average,z-score <2.0.There is a wide spectrum of aortic arch hypoplasia.And each patient has different anatomic and physiological characteristics.Aortic arch hypoplasia usually occurs in association with intra-cardiac anomalies.And its rates of complication,reintervention and mortality are unsatisfactory.The major surgical techniques are extended end-to-end anastomosis,subclavian flap approach,end-to-side anastomosis and patch aortoplasty.And the major perfusion techniques include deep hypothermic circuitry arrest and selective cerebral perfusion.A proper selection of surgical approaches and perfusion techniques may improve the outcomes.According to the specific status of aortic arch hypoplasia patient,individualized treatment is needed.Here we make a review of progress of surgery and perfusion technique in aortic arch hypoplasia.%目前,小儿主动脉弓发育不良的定义存在争议,主要的评价方法有:①近弓、远弓和峡部分别<升主动脉的60%、50%和40%、或者横弓直径<升主动脉远端的50%、或者横弓直径<降主动脉直径的50%;②经验法则:横弓直径(mm)<体重(kg)+1;③Z值:不同节段的大小低于正常平均值的两个标准差以下,即Z值<-2.小儿主动脉弓发育不良病变谱广,不同患儿解剖和生理特点差异大,且常合并各类心内畸形,小儿主动脉弓发育不良

  2. Aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant%自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

    Institute of Scientific and Technical Information of China (English)

    鲁亚南; 刘锦纷; 徐志伟; 苏肇伉; 丁文祥

    2011-01-01

    under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.

  3. High arch

    Science.gov (United States)

    Pes cavus; High foot arch ... High foot arches are much less common than flat feet. They are more likely to be caused ... difficult to fit into shoes. People who have high arches most often need foot support. A high ...

  4. Bicuspid Aortic Valve

    Science.gov (United States)

    2006-08-01

    aortic valvular disease, endocarditis, ascending aortic aneurysm and aortic dissection.1-4 There is also an association of BAV with coarctation of...for aortic aneurysm , patients with BAV appear to have additional risks for aortic disease. Nistri et al.12 reported significant aortic root...Congenital heart disease in patients with Turner’s syndrome. Italian study group for Turner syndrome (ISGTS). J Pediatr 1998; 133:688-692. 7. Schmid

  5. Surgical treatment of interrupted aortic arch associated with ventricular septal defect and patent ductus arteriosus in patients over one year of age

    Institute of Scientific and Technical Information of China (English)

    Li Zhiqiang; Li Bin; Fan Xiangming; Su Junwu; Zhang Jing; He Yan; Liu Yinglong

    2014-01-01

    Background Interrupted aortic arch (IAA) is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.However,in China,patients with IAA associated with ventricular septal defect (VSD) and patent ductus arteriosus (PDA) over one year of age are common.So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age.Methods From January 2009 to December 2012,19 patients with IAA have undergone complete single-stage repair.The patients' mean age was 4.4 years,ranging 1 to 15 years; and their mean weight was 12.8 kg,ranging 4.2 to 36.0 kg.Fifteen IAA were type A,four were type B.Preoperative cardiac catheterization data were available from all patients.Mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were measured.The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case.All patients underwent echocardiographic examinations before discharged from the hospital.In addition,cardiac catheterization and echocardiographic examinations were performed during follow-up.Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients.Mean follow-up was (1.6±0.8) years.Results There were two hospital deaths (2/19,11%).One patient died of pulmonary hypertension crisis,and another died of postoperative low cardiac output.Five cases had other main postoperative complications but no postoperative neurologic complications.Seventeen survivors were followed up,and there were no late deaths or reoperation.Mean cross-clamp duration was (85±22) minutes and selective brain perfusion duration was (34±11) minutes.Two patients required delayed sternal closure at two days postoperatively.Intensive care unit and hospital stays were (9±8) days and (47±24) days,respectively.Pressure gradients across

  6. Imaging Features of Angiocardiography in Interruption of Aortic Arch%主动脉弓离断的心血管造影影像分析

    Institute of Scientific and Technical Information of China (English)

    彭辽河; 魏崇健; 乔英; 闫小仿

    2011-01-01

    Objective To analyze the imaging features of angiography in interruption of aortic arch (IAA).Materials and Methods Eight patients underwent right and left ventricle angiography as well as descending aorta angiopraphy via the femoral artery and vein.The ascending aorta angiography was subsequently performed through the right carotid artery in 3 eases,and 3 patients performed ascending aorta angiography through the right ventrieular septal defect.Results No blood flow was detected between the ascending and descending aorta in all cases.The angiography revealed aorta stenosis in 7 cases,aorta expansion in 1 case,the link between the tip of descending aorta and the patent duetns arteriosns in 7 cases,the link between descending aorta and subclavian artery in 1 case.All the 8 cases coexisted with inner-heart malformation,including ventricular septal defect in 5, patent ductus arteriosus and pulmonary arterial hypertension in 2, double outlet of right ventricle and patent ductus arteriosus and pulmonary arterial hypertension in 2, tetralogy of Fallot in 1 case.Conclusion The angiocardiography is an important method for early diagnosis and surgical therapy strategy.%目的 分析主动脉弓离断(interruption of aortic arch,IAA)患者的心血管造影影像,提高对本病的认识和诊断准确性.资料与方法 8例患者均经股动脉、股静脉穿刺插管,行右心室、左心室、降主动脉造影.其中3例经右颈总动脉穿刺插管行升主动脉造影;3例从右心室经室间隔缺损至升主动脉造影.结果 8例升主动脉与降主动脉之间均无血流连续.升主动脉细小7例,升主动脉扩张1例,降主动脉顶端与未闭的动脉导管连接7例,降主动脉与右锁骨下动脉连接1例.8例均合并心内畸形,其中室间隔缺损+动脉导管未闭并肺动脉高压5例,右室双出口+动脉导管未闭并肺动脉高压2例,法乐四联症1例.结论 心血管造影是IAA确诊的重要方法,能够为IAA的早期有效

  7. 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.

  8. Diagnostic Value of Prospective Electrocardiogram-triggered Dual-source Computed Tomography Angiography for Infants and Children with Interrupted Aortic Arch

    Institute of Scientific and Technical Information of China (English)

    Hai-Ou Li; Xi-Ming Wang; Pei Nie; Xiao-Peng Ji; Zhao-Ping Cheng; Jiu-Hong Chen; Zhuo-Dong Xu

    2015-01-01

    Background:Accurate assessment of intra-as well as extra-cardiac malformations and radiation dosage concerns are especially crucial to infants and children with interrupted aortic arch (IAA).The purpose of this study is to investigate the value of prospective electrocardiogram (ECG)-triggered dual-source computed tomography (DSCT) angiography with low-dosage techniques in the diagnosis of IAA.Methods:Thirteen patients with suspected IAA underwent prospective ECG-triggered DSCT scan and transthoracic echocardiography (TTE).Surgery was performed on all the patients.A five-point scale was used to assess image quality.The diagnostic accuracy ofDSCT angiography and TTE was compared with the surgical findings as the reference standard.A nonparametric Chi-square test was used for comparative analysis.P<0.05 was considered as a significant difference.The mean effective radiation dose (ED) was calculated.Results:Diagnostic DSCT images were obtained for all the patients.Thirteen IAA cases with 60 separate cardiovascular anomalies were confirmed by surgical findings.The diagnostic accuracy of TTE and DSCT for total cardiovascular malformations was 93.7% and 97.9% (P>0.05),and that for extra-cardiac vascular malformations was 92.3% and 99.0% (P < 0.05),respectively.The mean score of image quality was 3.77 ± 0.83.The mean ED was 0.30 ± 0.04 mSv (range from 0.23 mSv to 0.39 mSv).Conclusions:In infants and children with IAA,prospective ECG-triggered DSCT with low radiation exposure and high diagnostic efficiency has higher accuracy compared to TTE in detection of extra-cardiac vascular anomalies.

  9. [Peritoneal dialysis in a patient with right hemiparesis, lupus nephritis, significant insufficiency of arteries of aortic arch and celiac disease: case report].

    Science.gov (United States)

    Altabas, Karmela; Crne, Natasa; Franjić, Björn Dario; Pavlović, Drasko; Josipović, Josipa

    2012-10-01

    Peritoneal dialysis (PD) is a method of choice in patients in whom there are difficulties concerning creation of AV fistula. A 38-year old female patient came to our hospital because of a need of making an AV fistula. She had end-stage renal insufficiency of unknown origin. She had a right hemiparesis with a contracture of the right fist and epilepsy because of the stroke she suffered in 1993. After doing the diagnostics, we have found that patient had lupus nephritis, occlusion of brachiocephalic trunk, right and left common carotid artery and left subclavian artery. We also diagnosed celiac disease and a significant anemia. It was not possible to form an AV fistula, as it was not possible to do an assisted PD. Because of the right hemiparesis and contracture of the right fist, the possibility of performing PD independently was questionable. Despite the handicap, the patient had strong motivation and she managed to master the technique of PD independently. Even though it was estimated that she had a high risk score for applying anesthesia (ASA IV), the insertion of the peritoneal catheter went without complications. Because of the comorbidity, especially because of the significant stenosis and occlusions of the arteries of aortic arch, the kidney transplantation will not be performed. In the last fifteen months, the patient is performing PD independently, without any infectious complications, she is feeling well and is satisfied with the quality of her life. The consequences of the renal insufficiency are under control, systemic lupus erythematosus is, with a low dose of corticosteroids, in a steady state, malnutrition is corrected, but there is still hypoalbuminemia noted.

  10. [Coarctation of the aorta with aortic arch hypoplasia in newborn with partial trisomy 11q associated to 4q interstitial deletion].

    Science.gov (United States)

    Palano, G M; Licata, F; Carpinato, C; Sottile, F; Sciuto, R; Mattina, T; Distefano, G

    2010-12-01

    This article reports the case of newborn with multiple dimorphisms (microcephaly, hypertelorism, wide and flat nasal bridge, small nose, long philtrum, microretrognathia, malformed and low-set ears, short neck, redundant nuchal skin, genital anomalies), admitted in the hospital after two days from delivery for torpor, poor food and cyanosis. Babies were affected, at color-Doppler echocardiography, by coarctation of the aorta (CoA) with aortic arch hypoplasia. CoA is often associated to genetic and environmental factors that interact frequently. In this study the anamnestic absence of teratogen noxae and the presence of facial and genital anomalies suggest a genetic study to provide appropriate genetic information to parents. G-banding chromosomic analysis revealed a 46, XX der 4t(4;11) karyotype with partial 11q trisomy confirmed with FISH chromosome painting 4;11 and with FISH subtelomere specific 4(p/q)11(p/q). These techniques showed that derivative chromosome 4 was constituted by chromosome 4 with partial deletion in the q35 region and by 11q21 translocation. This rare anomaly is often inherited by an unbalanced segregation of a balanced translocation, present in one of the two parents. In the present study, the father carried a t(4q;11q) balanced translocation. A CGH-array analysis was executed to the child for the breakpoints definition. As 11q trisomy cases reported in literature are still few, this case can contribute to improve our knowledge on the genotype-phenotype correlation in this rare genetic anomaly.

  11. A Case of Acute Aortic Dissection Type B Associated with Cushing's Syndrome

    Science.gov (United States)

    Petramala, Luigi; Cotesta, Dario; Sapienza, Paolo; Zinnamosca, Laura; Moroni, Enrico; di Marzio, Luca; De Toma, Giorgio; Letizia, Claudio

    2009-01-01

    We report a case of a 63-year-old man, with a previous history of hypertension and glucose intolerance associated troncular obesity that was emergently admitted to our Institution for evaluation of a severe, constant posterior chest pain which radiated anteriorly and dyspnoea with a suspected diagnosis of acute aortic dissection. A CT scan of thorax and abdomen demonstrated a dissection starting just below left succlavian artery and extending downward to the left renal artery, involving the celiac tripod and superior mesenteric artery. The dissection was classified as Stanford B, De Bakey III. Moreover, CT scan of abdomen revealed incidentally a left adrenal tumor of 25 mm of diameter. An emergent prosthetic graft was placed just below the origin of the left succlavian artery up-to the diaphragmatic hiatus. Furthermore, a diagnostic evaluation of the mass revealed an increase of cortisol production, and a diagnosis of Cushing's syndrome was done and the patient underwent an adrenalectomy via laparotomic approach. We report an association of acute aortic dissection of acute aortic dissection type B associated to Cushing's syndrome. Keywords Cushing's syndrome; Adrenocortical adenoma; Aortic dissection type B PMID:22505966

  12. Mutations in the TGF-β repressor SKI cause Shprintzen-Goldberg syndrome with aortic aneurysm.

    Science.gov (United States)

    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

    2012-11-01

    Elevated transforming growth factor (TGF)-β signaling has been implicated in the pathogenesis of syndromic presentations of aortic aneurysm, including Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS). However, the location and character of many of the causal mutations in LDS intuitively imply diminished TGF-β signaling. Taken together, these data have engendered controversy regarding the specific role of TGF-β in disease pathogenesis. Shprintzen-Goldberg syndrome (SGS) has considerable phenotypic overlap with MFS and LDS, including aortic aneurysm. We identified causative variation in ten individuals with SGS in the proto-oncogene SKI, a known repressor of TGF-β activity. Cultured dermal fibroblasts from affected individuals showed enhanced activation of TGF-β signaling cascades and higher expression of TGF-β-responsive genes relative to control cells. Morpholino-induced silencing of SKI paralogs in zebrafish recapitulated abnormalities seen in humans with SGS. These data support the conclusions that increased TGF-β signaling is the mechanism underlying SGS and that high signaling contributes to multiple syndromic presentations of aortic aneurysm.

  13. Fetal aortic root dilation: a prenatal feature of the Loeys-Dietz syndrome.

    Science.gov (United States)

    Viassolo, Valeria; Lituania, Mario; Marasini, Maurizio; Dietz, Harry; Benelli, Fabrizio; Forzano, Francesca; Faravelli, Francesca

    2006-11-01

    Loeys-Dietz syndrome is a recently described autosomal dominant disorder with cardinal manifestations in cardiovascular, craniofacial and skeletal systems. Although the disease has some phenotypic overlap with Marfan syndrome, the disease, that is caused by mutations in the transforming growth factor beta-receptor 1 (TGFBR1) or transforming growth factor beta-receptor 2 (TGFBR2) genes, presents many distinctive features and a particularly aggressive cardiovascular course. We describe prenatal identification of an aortic root aneurysm in a fetus of 19 week of gestation as an early marker of Loeys-Dietz syndrome.

  14. Tratamento cirúrgico dos aneurismas e dissecções do arco aórtico Surgical treatment of aneurysms and dissections of the aortic arch

    Directory of Open Access Journals (Sweden)

    Bayard GONTIJO FILHO

    1999-10-01

    Full Text Available No período de jan/1990 a mar/1999, foram realizados 354 operações para tratamento de lesões da aorta torácica em nosso Serviço. Destas, 47 foram relacionadas a procedimentos no arco aórtico; 31 (66,0% pacientes eram do sexo masculino e a idade variou de 26 a 74 anos (m = 54,9 ± 10,9. A dissecção aórtica foi responsável pela indicação cirúrgica em 33 (70,2% pacientes e os aneurismas fusiformes ou saculares em 14 (29,8%. A operação foi indicada em caráter de urgência em 10 (21,3% pacientes, havendo 4 casos com sinais de rotura; 11 (23,4% pacientes já haviam sido submetidos a operação cardíaca prévia. O acesso cirúrgico foi a esternotomia mediana (42 casos ou a toracotomia esquerda ou bilateral (5 casos. Hipotermia profunda com parada circulatória (PC foi empregada em 97,8% dos casos com o tempo PC variando de 15 a 60 minutos (m = 30,6 ± 12,6. A técnica do tipo hemiarco anterior foi empregada em 19 (40,4% pacientes, o hemiarco posterior em 5 (10,6%, a substituição total em 18 (38,3%, plastia em 4 (8,5% e derivação extra-anatômica em 1 (2,1%. Foram realizados os seguintes procedimentos concomitantes: substituição da aorta ascendente por conduto valvulado (15, revascularização miocárdica (9, tromba de elefante (5 e troca valvar aórtica (3. A mortalidade hospitalar foi de 12,3% (6 pacientes sendo a complicação não fatal mais freqüente a insuficiência respiratória (7 casos. A análise dos fatores determinantes de mortalidade hospitalar demonstraram tendência estatística para idade acima de 60 anos (p = 0,17 e para portadores de dissecção aguda (p = 0,07. Dos 41 pacientes que receberam alta, 39 foram seguidos por um período de 1 mês a 9 anos. Houve 3 óbitos, sendo 1 por AVC e 2 em reoperações, necessárias em 5 pacientes. A sobrevida de 9 anos é de 80,85%.From Jan/1990 to Mar/1999 we performed 354 operations on the thoracic aorta in our hospital. Among these, 47 procedures involved the aortic arch

  15. 快速成型技术制备仿生主动脉弓模型的研究%Preliminary study on rapid prototyping to prepare biomimetic model of aortic arch

    Institute of Scientific and Technical Information of China (English)

    陈鹏飞; 严中亚; 申运华; 陈向东; 邓克学

    2015-01-01

    目的 创建与人体解剖结构一致的仿真主动脉弓三维快速成型模型,探讨其对促进规划和执行主动脉弓外科手术的帮助,为构建个体化人工血管支架及3D打印生物组织或器官进行前期准备.方法 对正常成年男性进行胸部CT扫描,获得该男子主动脉弓原始CT数据(DICOM),通过Mimics 10.01软件对DICOM数据处理,分割出主动脉弓的区域,计算主动脉弓三维立体模型,导出三维模型数据文件(STL文件),最后通过3D打印机打印出主动脉弓三维模型.结果 基于CT获得的胸部解剖结构的数据,通过MIMICS 10.01软件进行处理并导出STL格式文件后,可直接用3D打印机打印出与人体主动脉弓解剖结构基本一致的主动脉弓模型(比例1∶1).结论 快速成型技术制备的主动脉弓模型对手术规划有临床意义,有助于构建个体化人工血管支架及3D打印生物组织或器官.%Objective To create an anatomical correct 3D rapid prototyping (RP) model for a volunteer' s aortic arch and investigate the significance of aortic arch model for surgical planning.To make the early preparations for the mannufacture of individualize artificial vascular stents and 3D printing with biological tissue or organ.Methods Based on the extracted 3D contour image from volunteer' s chest CT data,the custom-made artificial aortic arch was designed with mimics 10.01 software and form 3D printing STL file.And then a aortic arch model was printed out by 3D printer.Results The data acquired by CT scanner can be rapidly prototyped after being edited with Mimics 10.01 software and directly used by 3D printing system to construct a aortic arch model (1∶1).Conclusion The artificial 3D aortic arch model has significance in surgical planning and great help for the mannufacture of individualize artificial vascular stents and 3D printing with biological tissue or organ.

  16. 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.

  17. Aortic Valvuloplasty as Bridging for TAVI in High-Risk Patients with Heyde’s Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Cosmo Godino

    2012-01-01

    Full Text Available There is a frequent association between aortic valve stenosis and gastrointestinal bleeding, also known as Heyde’s syndrome. In these patients, the aortic valve replacement should be recommended as “gold standard.” In high-surgical-risk patients, the Transcatheter Aortic Valve Implantation (TAVI is an alternative option. However, the risk of bleeding recurrence, related to double antiplatelet therapy started after TAVI, cannot be excluded especially in the first months. We present a case of a patient with a severe aortic valve stenosis and a history of previously documented angiodysplasia and recurrence of gastrointestinal bleeding initially treated only with balloon aortic valvuloplasty that excluded recurrence of bleeding during the subsequent six months of followup. Therefore, a definite transfemoral Edwards XT valve implantation was planned to be performed in case of recurrence of aortic stenosis.

  18. Reoperation on aortic disease in patients with previous aortic valve surgery

    Institute of Scientific and Technical Information of China (English)

    SUN Xiao-gang; ZHANG Liang; YU Cun-tao; QIAN Xiang-yang; CHANG Qian

    2013-01-01

    Background Aortic valve replacement (AVR) is a safe and effective method in the treatment of aortic valve diseases.This study aimed to increase the understanding on re-treatment of aortic diseases after aortic valve surgery through a retrospective analysis of 47 related cases.Methods Forty-seven patients (38 males and 9 females) with previous aortic valve surgery have received reoperation on aorta from January 2003 to June 2012,and the mean interval time of re-intervention to aortic disease was 6 years ((6.0± 3.8) years).The secondary aortic surgery included aortic root replacement (14 cases),ascending aorta replacement (10 cases),aortic root/ascending aorta plus total arch replacement with stented elephant trunk implantation (21 cases),and total thoracoabdominal aorta replacement (2 cases).All these patients have received outpatient re-exams or follow-up by phone calls.Results After the initial aortic valve replacement,patients suffered from aortic dissection (25 cases,53%),ascending aortic aneurysm (12 cases,26%) or aortic root aneurysm (10 cases,21%).Diameter in ascending aorta increased (5.2±7.1) mm per year and aortic sinus (3.3±3.1) mm per year.The annual growth value of diameter in ascending aorta was higher in patients with rheumatic heart disease than that in Marfan syndrome (P<0.05).All 47 patients have received reoperation on aorta.One patient died in operating room because aortic dissection seriously involved right coronary artery.Seven patients had renal insufficiency after operation; neurological complications occurred in 14 patients including 7 patients with stroke and the others with transient brain dysfunction.All patients were followed up,the mean survival time was (97.25±17.63) months,95% confidence interval was 55.24-73.33 months.Eight cases were died during follow-up and five-year survival rate was 83%.Conclusion To reduce the aortic adverse events after first aortic valve surgery,it is necessary to actively treat and strictly

  19. 胎儿右位主动脉弓与染色体异常的相关性分析%Correlation between fetal right aortic arch and chromosome abnormality

    Institute of Scientific and Technical Information of China (English)

    刘锦平; 李亮; 王静

    2016-01-01

    目的:对系统超声检出胎儿右位主动脉弓与染色体异常的相关性进行分析。方法采集2009年1月至2014年12月在我院进行系统超声产前筛查19例右位主动脉弓胎儿,所有入选右位主动脉弓胎儿均进行羊水穿刺进行染色体核型分析,评价系统超声检测出的胎儿右位主动脉弓与染色体异常之间的相关性。结果15例右位主动脉弓超声像图表现气管和食管被气管左侧的动脉导管和气管后方的迷走左锁骨下动脉围成一“U”字形血管结构;1例胎儿有双主动脉弓超声主要表现为气管和食管被左右两侧的主动脉弓环绕成一个“O”字形包绕;3例右位主动脉弓胎儿另有左位动脉导管及头臂动脉影像分支,超声图主要表现为动脉导管在三血管-气管切面上并未有显示出来,气管的前方是动脉导管,未形成血管环;19例右位主动脉弓胎儿均进行染色体核型分析,3例伴室间隔缺损、单心房、三尖瓣闭锁、肺动脉狭窄,核型为18-三体;4例伴室间隔缺损、完全性房室通道、单心房、右室双出口、肺动脉狭窄近闭锁,核型为18-三体;3例伴单心室、单心房,核型为21-三体;1例法洛四联症和1例右位心、主动脉狭窄,伴有22q11.2。结论在产前胎儿超声筛查中要重视三血管-气管切面上的超声图像,有助于提高右位主动脉弓的检出率,胎儿右位主动脉弓与18-三体、21-三体等染色体病有明显相关性,若发现胎儿右位主动脉弓,应进行染色体核型进一步分析,排除染色体病变,从而达到优生优育的目的。%Objective To analyze the correlation between fetal right aortic arch and chromosome ab-normality by ultrasound.Methods From January Jan 2009 to Dec 2014,nineteen cases with fetal right aortic arch were enrolled.They were all determined by chromosome karyotype analysis.The correlation of fetal right aortic arch and chromosome abnormalities detected

  20. 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.

  1. Chronic type B aortic dissection in association with Hemolyticuremic syndrome in a child.

    Science.gov (United States)

    Gera, D N; Ghuge, P P; Gandhi, S; Vanikar, A V; Shrimali, J D; Kute, V B; Trivedi, H L

    2013-11-01

    Aortic dissection (AD) is a potentially life-threatening medical emergency usually encountered in the elderly. Here, we report a 9-year-old child who was incidentally detected to have asymptomatic chronic type B dissecting aneurysm of aorta when he presented with relapse of Hemolytic uremic syndrome (HUS) without any genetic abnormalities like Marfan or Ehler-Danlos syndrome. To the best of our knowledge, this is the first case of AD associated with HUS in a child without any known associated genetic or inherited risk factors.

  2. Thoracic aortopathy in Turner syndrome and the influence of bicuspid aortic valves and blood pressure: a CMR study

    Directory of Open Access Journals (Sweden)

    Hager Alfred

    2010-03-01

    Full Text Available Abstract Background To investigate aortic dimensions in women with Turner syndrome (TS in relation to aortic valve morphology, blood pressure, karyotype, and clinical characteristics. Methods and results A cross sectional study of 102 women with TS (mean age 37.7; 18-62 years examined by cardiovascular magnetic resonance (CMR- successful in 95, echocardiography, and 24-hour ambulatory blood pressure. Aortic diameters were measured by CMR at 8 positions along the thoracic aorta. Twenty-four healthy females were recruited as controls. In TS, aortic dilatation was present at one or more positions in 22 (23%. Aortic diameter in women with TS and bicuspid aortic valve was significantly larger than in TS with tricuspid valves in both the ascending (32.4 ± 6.7 vs. 26.0 ± 4.4 mm; p Conclusions Aortic dilatation was present in 23% of adult TS women, where aortic valve morphology, age and blood pressure were major determinants of the aortic diameter.

  3. [Neuroleptic malignant syndrome after aortic valve replacement; report of a case].

    Science.gov (United States)

    Ichiba, T; Takemoto, N

    2003-09-01

    A 64-year-old male with treated Parkinson's disease underwent mechanical valve replacement for aortic valve regurgitation. The antiparkinsonian drugs for internal use were interrupted on the morning of the operative day. After the operation, the patient developed fervescence, muscle rigidity, hidropoiesis and a rise in creatine kinase. The patient was diagnosed as neuroleptic malignant syndrome and given medication dantrolene sodium and antiparkinsonian drugs on the 5th postoperative day. The symptom of neuroleptic malignant syndrome disappeared on 12 postoperative days. As the stress of open heart surgery with extracorporeal circulation trigger off neuroleptic malignant syndrome, the patient with Parkinson's disease need early beginning of antiparkinsonian drugs on account of prevention of neuroleptic malignant syndrome after operation.

  4. 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.

  5. An incidentally discovered asymptomatic para-aortic paraganglioma with Peutz-Jeghers syndrome

    Directory of Open Access Journals (Sweden)

    Nazish Butt

    2012-01-01

    Full Text Available Peutz-Jeghers syndrome (PJS is an autosomal dominant inherited disorder characterized by mucocutaneous melanin pigmentation and gastrointestinal (GI tract hamartomatous polyps and an increased risk of malignancy. In addition to polyposis, previous studies have reported increased risk of GI and extraGI malignancies in PJS patients, compared with that of the general population. The most common extraintestinal malignancies reported in previous studies are pancreatic, breast, ovarian and testicular cancers.We report the case of a 17-year-old boy who presented with generalized weakness, recurrent sharp abdominal pain and melena, had exploratory laparotomy and ileal resection for ileo-ileal intussusception. Pigmentation of the buccal mucosa was noted. An abdominal computed tomography scan (CT revealed multiple polyps in small bowel loops. Gastroscopy revealed multiple dimunitive polyps in stomach and pedunculated polyp in duodenum. Colonoscopy revealed multiple colonic polyps. Pathological examination of the polyps confirmed hamartomas with smooth muscle arborization, compatible with Peutz-Jeghers polyps. CT scan guided left para-aortic lymph node biopsy revealed the characteristic features of extra-adrenal para-aortic paraganglioma. Although cases of various GI and extra GI malignancies in PJS patients has been reported, the present case appears to be the first in literature in which the PJS syndrome was associated with asymptomatic extraadrenal para-aortic paraganglioma. Patients with PJS should be treated by endoscopic or surgical resection and need whole-body screening.

  6. Mega aorta syndrome: a case of thoracic and abdominal aortic aneurysm.

    Science.gov (United States)

    Wu, William C; Mitchell, Christopher A; Linklater, Derek

    2010-07-01

    An 83-year-old woman presented to the emergency department (ED) via emergency medical services with the chief complaint of "strokelike symptoms." Physical examination revealed altered mental status, tachycardia, hypotension, and a large nonpulsatile periumbilical mass. Bedside ultrasound revealed a 9-cm abdominal aortic aneurysm with absent central flow. Computed tomography scan demonstrated diffuse thoracic and abdominal aortic dilation with rupture into the mediastinum along with left hemothorax. Repeat beside ultrasound demonstrated abdominal aortic aneurysm rupture not seen on the computed tomography scan. Despite aggressive resuscitation, the patient developed bradycardia, which devolved into pulseless electric activity cardiac arrest. She was unable to be resuscitated. The patient's diffuse aneurysmal dilation places her into the small category of patients with a disease entity known as mega aorta syndrome (MAS). Mega aorta syndrome is defined as aneurysmal dilation of the aorta to greater than 6 cm in diameter. Although not in our case, most cases of MAS are symptomatic before catastrophic presentation. The disease progression for these patients is slow and occurs over years. When this disease is recognized early, a surgery known as the elephant trunk procedure can be performed. This operation replaces the entire aorta in multiple stages. This gives the emergency physician a critical role in the diagnosis and outcome of these patients because they may come through the ED for an unrelated complaint early in the disease process. This case report illustrates an advanced case of MAS.

  7. The experience of cardiopulmonary bypass management of aortic arch surgery in neonates%新生儿主动脉弓手术的体外循环管理体会

    Institute of Scientific and Technical Information of China (English)

    吴永红; 孙善权; 姚仕文; 孔娟娟

    2015-01-01

    目的:探讨新生儿主动脉弓离断(IAA)或主动脉缩窄(CoA)合并其它心内畸形的一期矫治手术的体外循环管理。方法2014年9月至2015年7月手术治疗主动脉弓离断或缩窄合并其它心内畸形新生儿14例,其中主动脉缩窄6例,主动脉弓离断8例。平均年龄17(3~30) d。平均体重3.06(2.1~4.2)kg。均经胸骨正中切口在深低温体外循环下行一期矫治手术。结果14例新生患儿均顺利完成手术,顺利脱离体外循环,平均体外循环时间141(66~410) min,平均停循环时间17(15~30)min,平均阻断时间60(32~134)min,辅助时间69(28~284)min。其中有2例延迟关胸,14例手术开放后均尿量满意,无需进行改良超滤,无需放腹膜透析。结论新生儿的体外循环管理,尤其是深低温停循环的体外循环管理,从做好预充液的调整到体外循环的全程控制,会对手术结局起到非常重要的作用,患者有更好的转归。%Objective The aim of the study was to examine the management and the experience of cardiopulmonary bypass ( CPB) in one stage repair of interrupted aortic arch or aortic coarctation with intracardiac defects in neonates. Methods One stage re⁃pair were performed in 14 neonates with interrupted aortic arch( IAA) or aortic coarctation( CoA) with other intracardiac defects from September 2014 to July 2015. The cases included 6 neonates with aortic coarctation of aortic arch and 8 with IAA. Median age was 17 (3-30) days. Median weight was 3.06(2.1-4.2)kg. Results Deep hypothermia circulatory arrest(DHCA) was used in all cases. The median CPB time 141(66-410) min, median DHCA time was 17(15-30) min, and aortic cross clamping time was 60(32-134)min, and continuous CPB time 69(28-284)min. All the cases were weaned from CPB successfully without modified ultrafiltration and perito⁃neal dialysis. The postoperative complication occurred in 2 cases with

  8. 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.

  9. 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...

  10. AORTIC ANEURYSM: A RARE CAUSE OF ORTNER’S SYNDROME

    OpenAIRE

    2014-01-01

    A 60 year old male patient presented with hoarseness of voice lasting for 2 months. First he was examined by the otorhinolaryngologist of our hospital, who noticed absence of movements of the left vocal cord. Chest X-ray showed a large opacity in the left upper zone and the patient was referred to the pulmonologist. His general physical examination was unremarkable not having any stigmata of Marfan's or Ehler Danlos syndrome. On chest auscultation, a systolic flow murmur was ...

  11. Colaterais do arco aórtico no gambá (Didelphis albiventris Collaterals of the aortic arch in opossun (Didelphis albiventris

    Directory of Open Access Journals (Sweden)

    Sueli Hoff Reckziegel

    2003-06-01

    the right common carotid artery and the right subclavian artery. There was not a braquiocephalic trunk in 4 cases (14.28%, so it was first originated at the right subclavian artery and after the bicarotid trunk. The right subclavian artery and the right common carotid artery were originated from the braquiocephalic trunk in one case (3.57%, being the left common carotid artery originated directly from the aorta artery. The left subclavian artery originated directly from the aortic arch in all cases.

  12. 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

  13. 胸降主动脉瘤伴主动脉右弓右降手术的护理配合%Nursing cooperation in the operation of descending thoracic aortic aneurysm with right-sided aortic arch and right-sided descending aorta

    Institute of Scientific and Technical Information of China (English)

    李红; 翟跃军; 赵子英

    2010-01-01

    目的 探讨临床少见的右位主动脉弓、右位降主动脉、胸降主动脉瘤外科手术中的护理配合,以提高手术配合质量,减少手术相关并发症的发生.方法 针对8例行右弓、右降胸降主动脉瘤手术的病例进行术前准备、麻醉配合、体位管理和皮肤保护、术中配合、术后交接等护理配合.结果 8例患者于术后4~7 h清醒,无左上肢缺血、无皮肤压伤感染等护理问题.结论 术前心理护理、麻醉配合、正确的体位摆放以及充分显露术野和娴熟的手术配合是确保手术顺利进行的关键因素之一.%Objective To discuss the nursing cooperation in operation of right-sided aortic arch, rightsided descending aorta, descending thoracic aortic aneurysm, to provide basis for high quality of operative cooperation and complication reducing. Methods To carry out preoperative preparation, anesthesia cooperation, body position management, skin protection, operative cooperation, postoperative relay in 8 patients underwent operation of right-sided aortic arch, right-sided descending aorta, descending thoracic aortic aneurysm. Results 8 patients got consciousness 4 ~ 7 hours post operation, there were no left upper limb ischemia and compression injury infection. Conclusions Preoperative mental nursing, anesthesia cooperation,correct body position, sufficient surgery field exposure and expert operative cooperation are the key factors of success operation.

  14. Guyon's tunnel syndrome during pregnancy with concomitant anomalous arch of the ulnar nerve: a case report.

    OpenAIRE

    2014-01-01

    Numerous causes are reported for ulnar nerve compression at the wrist, known as Guyon's tunnel syndrome. In the present article, a patient with Guyon's tunnel syndrome during pregnancy concomitant with an anomaly of ulnar nerve is described. A 29-year-old Iranian woman presented with clinical features of Guyon's tunnel syndrome (pain and paresthesia in the fifth finger of the left hand and atrophy of the first dorsal interosseus muscle). Symptoms of the patient appeared during the third trime...

  15. 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.

  16. Angiograms of the Abdominal Aorta in a Patient of Middle Aortic Syndrome

    Institute of Scientific and Technical Information of China (English)

    Zhou Yafeng; Yang Xiangjun; Song Jianping; Jiang Tingbo; Li Xun; Hui Jie; Liu Zhihua; Jiang Wenping

    2007-01-01

    A 42-year old woman of middle aortic syndrome presented with severe hypertension for 20 years, whose abdominal aorta angiography revealed long segment and nearly complete occlusion in the mid portion of abdominal aorta, and extensively enlarged collateral vascular supply to the lower portion of abdominal aorta. The pressures proximal and distal to stenosis were 185/110 and 95/70 mmHg, and the pressure gradient across the stenosis was 90/40 mmHg. After the operation of thoraco-abdominal bypass graft, the pressure difference between the upper and lower extremities eventually disappeared.

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

    DEFF Research Database (Denmark)

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

    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......-average=-0.312 and -0.341; pperturbed sympatho-vagal balance is present in TS explained by a decreased vagal activity...

  18. Pacing to treat low cardiac output syndrome following elective aortic valve replacement

    Directory of Open Access Journals (Sweden)

    Muhammad Ishaq

    2012-01-01

    Full Text Available We report a case of low cardiac output syndrome caused by dynamic left ventricular (LV outflow obstruction after aortic valve replacement (AVR. This recognized phenomenon probably occurs more frequently than appreciated, and the author suggests that this should be considered when managing patients with severe hemodynamic instability after AVR. In addition, we also focus on the fact that invasive pacemaker systems have significant effects on cardiac output augmentation postoperatively and in long-term management of patients with LV outflow tract (LVOT obstruction following AVR. The possible mechanisms and subsequent treatments are discussed.

  19. Long-term implications of emergency versus elective proximal aortic surgery in Marfan syndrome patients in the GenTAC Registry

    Science.gov (United States)

    Song, Howard K.; Kindem, Mark; Bavaria, Joseph E.; Dietz, Harry C.; Milewicz, Dianna M.; Devereux, Richard B.; Eagle, Kim A.; Maslen, Cheryl L.; Kroner, Barbara L.; Pyeritz, Reed E.; Holmes, Kathryn W.; Weinsaft, Jonathan W.; Menashe, Victor; Ravekes, William; LeMaire, Scott A.

    2011-01-01

    Objective Marfan syndrome patients with aortic root aneurysms undergo elective aortic root replacement to avoid the life-threatening outcomes of aortic dissection and emergency repair. The long-term implications of failed aortic surveillance leading to acute dissection and emergency repair are poorly defined. We compared the long-term clinical courses of Marfan syndrome patients who survive emergency versus elective proximal aortic surgery. Methods The GenTAC Registry is an NIH-funded, multicenter database and biorepository that enrolls patients with genetically triggered thoracic aortic aneurysms. Of the 635 patients with Marfan syndrome enrolled as of March 2011, 194 had undergone proximal aortic replacement. Patients were grouped according to emergent (n=47) or elective (n=147) status at the time of surgery. Results Patients in the emergent group were more likely to have incomplete proximal aortic resection; 83% of emergency procedures included aortic root replacement, compared with 95% of elective procedures. At long-term follow-up (mean, >6 years), emergent patients had a higher incidence of chronic dissection of the distal aorta and had significantly larger diameters in distal aortic segments than elective patients. Additionally, emergent patients had undergone more operations (1.31 vs 1.11 procedures/patient; P=0.01) and had lower activity scores on a health-related quality-of-life survey. Conclusions For Marfan syndrome patients, failed aortic surveillance and consequent emergency dissection repair have important long-term implications with regard to status of the distal aorta, the need for multiple procedures, and quality of life. These findings emphasize the importance of aortic surveillance and timely elective aortic root aneurysm repair for Marfan syndrome patients. PMID:22104675

  20. Ultrasound studying in ascending aorta and aortic arch plaque in patients with the stroke%脑卒中患者升主动脉及主动脉弓斑块的超声研究

    Institute of Scientific and Technical Information of China (English)

    隗冬梅; 何文; 程晓雯; 刘晓峰

    2012-01-01

    目的 探讨利用超声检查脑卒中患者升主动脉和主动脉弓斑块的意义.方法 52例经核磁共振或电子计算机X射线断层扫描技术证实脑内存在急性或亚急性栓塞灶的患者,经食管和经胸超声心动图分别检测升主动脉和主动脉弓.了解出现在升主动脉和主动脉弓的斑块情况.结果 经胸超声心动图发现斑块位于升主动脉者18例,共20个斑块;经食管超声心动图发现斑块位于升主动脉者23例,共27个斑块.经胸和经食管超声心动图同时发现斑块位于升主动脉者16例,共18个斑块,其中不稳定斑块5个.经胸超声心动图发现斑块位于主动脉弓者29例,共43个斑块;经食管超声心动图发现斑块位于主动脉弓者40例,共58个斑块.经胸和经食管超声心动图同时发现斑块位于主动脉弓者25例,共35个斑块,其中不稳定斑块18个.经食管超声心动图发现的斑块数量多于经胸超声心动图.结论 结合经食管超声心动图,可以避免经胸超声心动图图像显示不清的缺点,提高急性脑卒中患者大血管病变的阳性检出率,并有助于斑块性质的描述,为急性脑卒中患者血管源性栓子来源提供依据.%Objective To investigate the plaque in ascending aorta and aortic arch by ultrasound in patients with the stroke. Methods Fifty-two patients with magnetic resonance ( MR) or X-ray computer tomography (CT) confirmed the presence of acute or subacute stroke, looking from the ascending aorta and aortic arch of vascular plaque by transesophageal and transthoracic echocardiography. The ascending aorta and aortic arch plaques were detected. Results There were 18 cases with a total of 20 plaques in the ascending aorta found by transthoracic echocardiography. There were 23 cases with a total of 27 plaques in the ascending aorta found by transesophageal echocardiography. There were 16 cases with a total of 18 plaques in the ascending aorta found by transthoracic and

  1. Aortic tear and dissection related to connective tissues abnormalities resembling Marfan syndrome in a Great Dane.

    Science.gov (United States)

    Lenz, Jennifer A; Bach, Jonathan F; Bell, Cynthia M; Stepien, Rebecca L

    2015-06-01

    Aortic tears and acute aortic dissection are rarely reported in dogs. This report describes a case of aortic dissection and probable sinus of Valsalva rupture in a young Great Dane with associated histopathologic findings suggestive of a connective tissue abnormality.

  2. Characterization of the inflammatory cells in ascending thoracic aortic aneurysms in patients with Marfan syndrome, familial thoracic aortic aneurysms and sporadic aneurysms

    Science.gov (United States)

    He, Rumin; Guo, Dong-Chuan; Sun, Wei; Papke, Christina L.; Duraisamy, Senthil; Estrera, Anthony L.; Safi, Hazim J.; Ahn, Chul; Buja, L. Maximilian; Arnett, Frank C.; Zhang, Jingwu; Geng, Yong-Jian; Milewicz, Dianna M.

    2008-01-01

    Objectives This study sought to characterize the inflammatory infiltrate in ascending thoracic aortic aneurysm (TAAs) in patients with Marfan syndrome (MFS), familial TAA (FTAA), and non-familial TAA cases. Background TAAs are associated with a pathologic lesion termed medial degeneration, which was described as a noninflammtory lesion. TAAs are a complication of MFS and also can be inherited in an autosomal dominant manner of FTAA. Methods Full aortic segments were collected from patients undergoing elective repair with MFS (n=5), FTAA (n=6) and TAAs (n=9), along with control aortas (n=5). Immunohistochemistry staining was performed using antibodies directed against markers of lymphocytes and macrophages. Real-time PCR analysis was performed to quantify the expression level of T cell receptor β chain variable region gene. Results Immunohistochemisty of TAA aortas demonstrated that the media and adventitia from MFS, FTAA and sporadic cases had increased numbers of T lymphocytes and macrophages when compared with control aortas. The number of T cells and macrophages in the aortic media of the aneurysm correlated inversely with the patient’s age at the time of prophylactic surgical repair of the aorta. Surprisingly, T cell receptor profiling indicated a similar clonal nature of the T cells in the aortic wall in a majority of aneurysms, whether the patient had MFS, FTAA or sporadic disease. Conclusion These results indicate that infiltration of inflammatory cells contributes to the pathogenesis of TAAs. Superantigen-driven stimulation of T lymphocytes in the aortic tissues of the TAA patients may contribute to the initial immune response. Ultramini-Abstract This study sought to investigate the infiltration of T-lymphocytes and macrophage in the aortas of patients with MFS, FTAA and sporadic TAAs. The results indicate that infiltration of inflammatory cells contributes to the pathogenesis of TAAs and superantigen-driven stimulation of T-lymphocytes may contribute to

  3. [Case of neuroleptic malignant syndrome following open heart surgery for thoracic aortic aneurysm with parkinson's disease].

    Science.gov (United States)

    Shinoda, Maiko; Sakamoto, Mik; Shindo, Yuki; Ando, Yumi; Tateda, Takeshi

    2013-12-01

    An 80-year-old woman with Parkinson's disease was scheduled for open heart surgery to repair thoracic aortic aneurysm. Parkinson's symptoms were normally treated using oral levodopa (200 mg), selegiline-hydrochloride (5 mg), bromocriptine-mesilate (2 mg), and amantadine-hydrochloride (200 mg) daily. On the day before surgery, levodopa 50mg was infused intravenously. Another 25 mg of levodopa was infused immediately after surgery. Twenty hours later, the patient developed tremors, heyperventilation, but no obvious muscle rigidity. Two days after surgery, the patient exhibited high fever, hydropoiesis, elevated creatine kinase, and a rise in blood leukocytes. She was diagnosed with neuroleptic malignant syndrome. She was intubated, and received dantrolene sodium. Symptoms of neuroleptic malignant syndrome disappeared on the fourth postoperative day. The stress of open heart surgery, specifically extracorporeal circulation and concomitant dilution of levodopa, triggered neuroleptic malignant syndrome in this patient. Parkinson's patients require higher doses of levodopa prior to surgery to compensate and prevent neuroleptic malignant syndrome after surgery.

  4. Medical image of the week: aortic ring

    Directory of Open Access Journals (Sweden)

    Wong C

    2014-10-01

    Full Text Available No abstract available. Article truncated after 150 words. A 78 year old man presented with altered mental status and was found to have an intraventricular hemorrhage. He was intubated for airway protection. On the post-intubation chest radiograph (Figure 1, the patient was noted to have a widening of the right paratracheal stripe. A CT chest (Figure 2 was obtained to characterize this finding and revealed an aortic ring which encircles the trachea and esophagus. Vascular rings are uncommon congenital abnormalities, accounting for approximately 1% of congenital heart disease. Complete vascular rings can occur with a right aortic arch with a ligamentum arteriosum or with a double aortic arch, such as with our patient (1. This ring can cause airway compression, stridor, esophageal compression, or no symptoms at all. As the embryo develops, the left fourth pharyngeal arch normally persists to become the aortic arch while the right fourth pharyngeal arch regresses. If both fourth pharyngeal arches persist, a ...

  5. Impact of aortic aneurysm on hospitalizations in patients with marfan syndrome: a multi-institutional study.

    Science.gov (United States)

    Collins, R Thomas; Phomakay, Venusa; Zarate, Yuri A; Tang, Xinyu

    2015-01-01

    Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting 1 in 3,000 people. Cardiovascular involvement is a prominent feature of MFS, with aortic dissection and/or rupture being the leading cause of death. Advances in the medical and surgical care of patients with MFS have improved survival. Hospital resource utilization and outcomes have not been evaluated in a large population of patients with MFS. We sought to analyze pediatric hospital resource utilization and outcomes in patients with MFS. Nationally distributed data from 43 pediatric hospitals in the 2004-2011 Pediatric Health Information System database were used to identify patients admitted to the hospital with International Classification of Diseases-9th Revision codes for a diagnosis of MFS. Aortic aneurysm (AA) with or without dissection, length of stay (LOS), and hospital charges were determined. During the study period, there were 1,978 admissions in 1,228 patients with MFS. AA was present in 217 (11%) admissions in 188 (15%) patients (63% male). Mean age of patients with AA was 13.8 ± 5.9 years. Aortic dissection or rupture was present in 15 (7% with AA) admissions in 15 (8% with AA) patients (mean age 15.7 ± 5.2 years). Other cardiac diagnoses occurred more commonly in the AA cohort (p < 0.0001), regardless of the reason for admission. Cardiothoracic surgical procedures were performed in 116 AA admissions (53%). Mean LOS, hospital charges per admission, and charges per day were significantly higher in AA cohort compared to those without AA. In-hospital mortality for AA was 2%. The presence of AA in patients with MFS increases hospital resource utilization. Cardiothoracic surgeries are commonly performed in this cohort. Other cardiovascular diagnoses are more prevalent in patients with AA suggesting a more severe phenotype.

  6. Bentall术联合全弓置换及支架象鼻手术治疗危重Debakey Ⅰ型主动脉夹层动脉瘤%Bentall operation combined with total arch replacement and stented elephant trunk implantation for serious Debakey I aortic dissecting aneurysm

    Institute of Scientific and Technical Information of China (English)

    谷天祥; 王春; 张玉海; 修宗谊; 房勤; 喻磊; 师恩祎

    2008-01-01

    Objective To summarize the clinical experience of Bentall operation combined with total arch replacement and stented elephant trunk implantation for serious Debakey I aortic dissecting aneurysrm Methods Twelve patients with serious Debakey I aortic dissecting aneurysm underwent surgical treatment from January 2005 to December 2007.There were 10 male and 2 female with the mean age of (40.1±9.5)years old.There were acute aortic dissection in 9 cases,chronic aortic dissection in 3 cases.The inner diameter of aorta was(5.3±1.8)cm. There were Mafan syndrome in 4 cases,aortic regurgitation in all cases,severely persistent chest pain in 9 eases,acute left heart failure in 8 cases,and cardiac tamponade in 4 cases.Bentall operations eombined with total arch replacement and stented elephant trunk implantation were performed by using deep hypothermic circulatory arrest and antegrade selective cerebral perfusion in all cases.Results Urgent surgery underwent in 9 cases.The mean interval between the onset of aortic dissection and the accomplishment of surgery wag(41.0±15.9)hours.Cardiopulmonary bypass time was(191±26)min,average cross clamp time was(134±31)min,and average deep hypothermie circulatory arrest time was(50.0±14.5)min One patient died in hospital.The time stayed in ICU was 3 to 27 d Mental disorder in 6 eases,hemiparalysis in 1 case,amputation in 1 case,hemorrhage of anastomosis in 1 ease.hemorrhage of alimentary tract in 1 cage,and pleural effusion in 4 cases were recorded.Eleven cases were followed-up for 8 weeks to 36 months.There were no bending of the stents and no obstruction in the vascular prosthesis.No re-operation was needed.One case died 6 months postoperatively.Conclusion Bentall operation combined with total arch replacement and stented elephant trunk implantation is safe and effective for serious Debakey I aortic dissecting aneurysm,while good organs protection and consummate cardiopulmonary bypass were taken.%目的 总结Bentall术同期全弓

  7. Risk Factor Analysis of Delayed Recovery of Consciousness after Aortic Arch Surgery%主动脉弓部手术后苏醒延迟的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    崔辉敏; 李庆国; 王强; 王东进

    2013-01-01

    Objective To determine risk factors of delayed recovery of consciousness after aortic arch surgery under deep hypothermic circulatory arrest (DHCA) and antegrade selective cerebral perfusion (ASCP). Methods We retrospectively analyzed clinical data of 113 patients who underwent aortic arch surgery under DHCA+ASCP in the Affiliated Drum Tower Hospital, Medical School of Nanjing University from October 2004 to April 2012. According to whether they regained consciousness within 24 hours after surgery, all the 113 patients were divided into normal group (73 patients including 55 males and 18 females with their average age of 48.1 ± 10.9 years) and delayed recovery group (40 patients including 29 males and 11 females with their average age of 52.2 ±11.4 years). Risk factors of delayed recovery of consciousness after surgery were evaluated by univariate analysis and multivariate logistic regression analysis. Results Nine patients (8.0%) died postoperatively, including 5 patients with multi-organ failure, 2 patients with heart failure, 1 patient with mediastinal infection, and 1 patient with pulmonary hemorrhage. There were 7 deaths (17.5%) in the delayed recovery group and 2 deaths (2.7%) in the normal group, and the in-hospital mortality of the delayed recovery group was significant- ly higher than that of the normal group (P=0.016). A total of 94 patients (including 65 patients in the normal group and 29 patients in the delayed recovery group) were followed up for 4-95 months. Eight patients (including 5 patients in the normal group and 3 patients in the delayed recovery group) died during follow-up, including 2 patients with stroke, 3 patients with heart failure, 2 patients with pulmonary hemorrhage and 1 patient with unknown cause. Ten patients were lost during follow-up. Univariate analysis showed that age(P=0.042), hypertension(P=0.017), emergency surgery (P=0.001), cardiopu-lmonary bypass (CPB) time (P=0.007), aortic cross-clamp time (P=0.021), and blood

  8. Endovascular stent-graft treatment of thoracic aortic syndromes: A 7-year experience

    Energy Technology Data Exchange (ETDEWEB)

    Dialetto, Giovanni [Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, V. Monaldi Hospital, Naples (Italy); Reginelli, Alfonso; Cerrato, Marcella [Department of Radiology, Second University of Naples, Naples (Italy); Rossi, Giovanni [Department of Radiology, Monaldi Hospital, Naples (Italy); Covino, Franco Enrico; Manduca, Sabrina [Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, V. Monaldi Hospital, Naples (Italy); Lassandro, Francesco [Department of Radiology, Monaldi Hospital, Naples (Italy)], E-mail: f.lassandro@tiscali.it

    2007-10-15

    Thoracic aortic diseases (TAD) are relatively frequent conditions associated with high mortality. Recently, several reports have demonstrated the safety and efficacy of endovascular stent-graft (EVG) placement for TAD as an alternative to open surgery. We report our experience in management of thoracic aortic syndrome on 56 consecutive patients with TAD that underwent endovascular stent-graft repair. MDCT angiography was used in all patients to provide preprocedure evaluation and measurements. In particular it is necessary to evaluate the proximal and distal landing zones of the stent-graft. All EVGs in our series were placed successfully. Conversion to open surgery was never required. Six patients (10.7%) died early after the stent-graft deployment. During follow-up four more patients died. The endoleak rate was 16.7% (no. 10 pt). We did not observe any case of paraplegia. The present study shows the efficacy of EVG in the long-term follow-up, with an overall survival of 82.1%, which is comparable to that reported in recent studies. In conclusion this technique is emerging as an alternative approach in the treatment of TAD because this approach offers a less invasive therapeutic option to standard surgical techniques, even in patients who have associated diseases that make them poor surgical candidates.

  9. Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations.

    Science.gov (United States)

    Turtle, E J; Sule, A A; Webb, D J; Bath, L E

    2015-07-01

    There is a general lack of awareness of the risk of aortic dissection in Turner syndrome (TS) from both patients with TS and their physicians. Patients often ignore symptoms for up to 24 h before seeking medical advice, significantly increasing their risk of death. A clinical profile of those at risk of dissection is emerging and includes the presence of congenital heart defects, aortic dilatation and hypertension. MRI has revolutionised the visualisation of cardiovascular anatomy in TS but remains underutilised, especially in children and adolescents, and there is currently little guidance on blood pressure (BP) assessment or hypertension management. Children and adolescents with TS at risk of dissection could be easily identified by timely imaging and BP assessment. This would allow medical management or surgical intervention to be put in place to reduce the risk of this major, and often fatal, complication. Since guidance is lacking, we have reviewed the literature on the risk factors for dissection in TS during childhood and adolescence, and make recommendations on the assessment and management of these patients.

  10. Hybrid Endovascular Aorta Repair with Simultaneous Supra-aortic Branch or Iliac Branch Revascularization

    Institute of Scientific and Technical Information of China (English)

    Yue-hong Zheng; Nim Choi; Hong-ru Deng; CU Kouk; Kun Yu; Furtado Rui

    2009-01-01

    Objective To describe a hybrid endovascular procedure for aorta repair with different kinds of bypass followed by concomitant placement of stent graft in the aorta.Methods From June 2007 to May 2008,5 consecutive patients who presented with aortic aneurysm or dissection were treated with a new hybrid aorta repair technique.Complete surgical rerouting of supra-aortic vessels was simultaneously created by endovascular repair of aortic arch aneurysm with stent graft.Hybrid left carotid-subclavian bypass with stent graft deployment covering the ostium of the left subclavian artery was performed in a Debakey type Ⅲ aortic dissection case.The supra-aortic branch was revascularized in 2 cases from ascending aorta to bilateral common carotid arteries using a 16-8 mm bifurcated graft,then total aortic arch and descending artery was occluded with stent-graft.The left carotid artery to the left subclavian artery bypass was created in 1 case,followed by stent-graft deployment.Two cases of infrarenal abdominal aortic aneurysm underwent left external iliac artery to left internal iliac artery bypass by a retroperineal route,then hybrid procedure was performed with bifurcated stent-graft.All stent grafts were deployed via a retrograde femoral artery approach in 5 patients.Results Technical success with complete aneurysmal exclusion was achieved in all patients.There was no incidence of endoleak.During a follow-up period of 2 to 10 months,documented perioperative neurologic events did not occur in all patients.One patient suffered from adult respiratory distress syndrome.After received tracheostomy,he recovered later.There was one death resulting from a postoperative myocardial infarction.Conclusion Hybrid arch repair provides an alternative therapy to patients otherwise considered prohibitively high risk for traditional open arch and thoracoabdominal aorta repair.

  11. 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.

  12. High-risk pregnancy in a woman with Marfan syndrome, a bicuspid aortic valve, and a dilated aortic sinus

    DEFF Research Database (Denmark)

    Groth, Kristian Ambjørn; Greisen, Jacob Raben; Nielsen, Birgitte Bruun;

    2015-01-01

    /day) to reduce blood pressure and was admitted for bed rest from week 30. Her aortic diameter was assessed by echocardiography every 2nd week and remained unchanged. She was treated with betamethason at week 26 and the child was born by a caesarean section in week 35. The post-operative course was uneventful....

  13. Ortner’s syndrome: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Bruno Landim Dutra

    2015-08-01

    Full Text Available AbstractThe authors report the case of a 55-year-old female, hypertensive, smoker patient presenting with dysphonia, dysphagia and persistent dry cough. Laryngoscopy diagnosed left vocal cord paralysis. Computed tomography demonstrated saccular aneurysm of the inferior wall of the aortic arch, stretching the left recurrent laryngeal nerve, a finding compatible with Ortner’s syndrome.

  14. The role of prostacyclin in the mesenteric traction syndrome during anesthesia for abdominal aortic reconstructive surgery.

    Science.gov (United States)

    Gottlieb, A; Skrinska, V A; O'Hara, P; Boutros, A R; Melia, M; Beck, G J

    1989-03-01

    Mesenteric traction syndrome consists of sudden tachycardia, hypotension, and cutaneous hyperemia, and frequently occurs during mesenteric traction in patients undergoing abdominal aortic aneurysm (AAA) reconstructive surgery. The etiology and clinical impact of this phenomenon are unknown, but the symptoms suggest a release of vasoactive materials from the mesenteric vascular bed. Thirty-one patients who underwent AAA surgery were studied. Mesenteric traction was accompanied by a decrease in systolic (p = 0.005) and diastolic (p less than 0.05) blood pressures, and in systemic vascular resistance (p less than 0.005), and was accompanied by an increase in heart rate (HR) (p less than 0.005), and cardiac output (p = 0.01). These hemodynamic changes coincided with an increase (p less than 0.001) in plasma concentrations of 6-keto-prostaglandin F1 (6-K-PGF1). No apparent change was found in prostaglandin E2, thromboxane B2, and histamine concentrations. The concentration of 6-K-PGF1 was correlated with diastolic blood pressure (r = -0.52, p less than 0.005) and HR (r = 0.65, p less than 0.001). Cutaneous hyperemia was observed in 58% of the patients. In an additional six patients, who had taken aspirin daily before AAA surgery, no significant changes were observed in the hemodynamic measurements or 6-K-PGF1 concentrations. These data suggest that mesenteric traction syndrome may be mediated at least in part by a selective release of prostacyclin.

  15. 停循环和局部脑灌在主动脉弓中断手术中的应用%The usefulness of deep hypothermic circulatory arrest and regional cerebral perfusion in interrupted aortic arch

    Institute of Scientific and Technical Information of China (English)

    郭铮; 王伟; 张蔚; 沈佳; 唐嘉忠; 朱德明

    2013-01-01

    Objective To evaluate the effects of deep hypothermic circulatory arrest (DHCA) and regional cerebral perfusion (RCP) in interrupted aortic arch (IAA) corrective surgery.Methods Thirty-one infants younger than 3-month-old with IAA were undergone corrective surgery with DHCA or RCP.The bypass time,aortic clamp time,DHCA or RCP time,hematocrit,ventilation time,ICU stay time and post-operative complications were recorded and compared between two groups.Results The bypass time,aortic clamp time were longer in RCP group,and the RCP time was longer than DHCA time.One postoperative death and two neurological complications took place in DHCA group,while no mortality and neurological complications in RCP group.There were no significant differences in mortality and morbidity between two groups.Conclusion Because complicated surgery is undergone in IAA corrective procedure,RCP technique is safer to protect the brain function.%目的 比较深低温停循环(DHCA)与局部低流量脑灌(RCP)在小儿主动脉弓中断(IAA)纠治手术中的应用效果.方法 总结31例3个月以下的IAA合并室间隔缺损、房间隔缺损的婴儿手术病例,分为DHCA和RCP两组不同的体外循环方式,对术中转流时间,阻断时间,红细胞比容,停循环或脑灌时间,呼吸机插管时间,ICU滞留时间,术后低心排出量,肾功能衰竭,肺部炎症,出凝血障碍,多器官功能障碍,神经系统并发症,院内死亡率等情况进行分析.结果 RCP组手术的体外循环时间,阻断时间及脑灌时间显著长于DHCA组.DHCA组患儿死亡1例,神经系统并发症2例,RCP组无死亡和神经系统并发症,但两组无统计学差异.结论 IAA手术需要较长时间在主动脉上进行操作,采用RCP技术可以更好的保护神经系统功能,减少并发症.

  16. Estratégia cirúrgica na transposição das grandes artérias associada à obstrução do arco aórtico Surgical strategy in transposition of the great arteries with aortic arch obstruction

    Directory of Open Access Journals (Sweden)

    Bayard Gontijo Filho

    2007-06-01

    Full Text Available OBJETIVO: Analisar nossa experiência no tratamento cirúrgico da transposição das grandes artérias (TGA associada à obstrução do arco aórtico. MÉTODO: Entre janeiro de 1998 e dezembro de 2005, realizamos 223 operações de Jatene para correção de TGA: 21 (9,4% pacientes apresentavam obstruções do arco aórtico. A anatomia do arco aórtico evidenciou: coarctação da aorta localizada (n=10; coarctação com hipoplasia tubular do arco aórtico (n=6; interrupção do arco aórtico (n=5. Comunicação interventricular (CIV: 19 pacientes (90,5%, sendo 11 do tipo Taussig-Bing. Desproporção importante entre aorta e artéria pulmonar e anomalias coronárias foram achados freqüentes. Houve 7 correções em dois estágios e 14 correções em um único estágio. A reconstrução do arco foi realizada por ressecção e anastomose término-terminal ampliada (13 ou por translocação da aorta ascendente (8. RESULTADO: Houve cinco (23,8% óbitos hospitalares; apenas um (11,1% nos últimos nove casos consecutivos. Reoperações no período hospitalar: revisão de hemostasia (5, CIV residual + coarctação não identificada (1, estenose residual de arco aórtico (1. Após a alta, houve dois óbitos e três pacientes foram submetidos a reintervenções para estenose da via de saída do ventrículo direito. CONCLUSÃO: O tratamento da transposição das grandes artérias associada à obstrução do arco aórtico apresenta alta complexidade e morbi-mortalidade. Empregamos as correções em um e em dois estágios, obtendo resultados comparáveis. Nossa preferência atual é pela correção precoce em um único estágio para todos os pacientes, independente de sua configuração anatômica.OBJECTIVE:To analyze our experience in the surgical correction of transposition of the great arteries associated with aortic arch obstruction. METHOD: From January 1998 to December 2005 we performed 223 arterial switch operations for transposition of the great

  17. Chronobiology of Acute Aortic Dissection in the Marfan Syndrome (from the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions and the International Registry of Acute Aortic Dissection).

    Science.gov (United States)

    Siddiqi, Hasan K; Luminais, Steven N; Montgomery, Dan; Bossone, Eduardo; Dietz, Harry; Evangelista, Arturo; Isselbacher, Eric; LeMaire, Scott; Manfredini, Roberto; Milewicz, Dianna; Nienaber, Christoph A; Roman, Mary; Sechtem, Udo; Silberbach, Michael; Eagle, Kim A; Pyeritz, Reed E

    2017-03-01

    Marfan syndrome (MFS) is an autosomal dominant connective tissue disease associated with acute aortic dissection (AAD). We used 2 large registries that include patients with MFS to investigate possible trends in the chronobiology of AAD in MFS. We queried the International Registry of Acute Aortic Dissection (IRAD) and the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) registry to extract data on all patients with MFS who had suffered an AAD. The group included 257 patients with MFS who suffered an AAD from 1980 to 2012. The chi-square tests were used for statistical testing. Mean subject age at time of AAD was 38 years, and 61% of subjects were men. AAD was more likely in the winter/spring season (November to April) than the other half of the year (57% vs 43%, p = 0.05). Dissections were significantly more likely to occur during the daytime hours, with 65% of dissections occurring from 6 a.m. to 6 p.m. (p = 0.001). Men were more likely to dissect during the daytime hours (6 a.m. to 6 p.m.) than women (74% vs 51%, p = 0.01). These insights offer a glimpse of the times of greatest vulnerability for patients with MFS who suffer from this catastrophic event. In conclusion, the chronobiology of AAD in MFS reflects that of AAD in the general population.

  18. 主动脉弓三分支覆膜支架在Stanford A型急性主动脉夹层外科治疗中的应用%The application of triple branches aortic arch stent-graft placement in the surgical treatment of acute Stanford type A aortic dissection

    Institute of Scientific and Technical Information of China (English)

    华菲; 沈振亚; 余云生; 叶文学; 黄浩岳

    2011-01-01

    was transected near the base of the innominate artery.From the incision, the triple-branched stent graft was implantated into the true lumen of the arch,descending aorta and the aorta bifurcation vessel. The transected stump of the ascending aorta was anastomosis to the proximal of the branched blood vessel prosthesis.Results Cardiopulmonary bypass time was (186 ±38) min,cross clamp time was (101 ±27) min,and average selective cerebral perfusion and lower body arrest time was ( 39 ± 11 ) min.The in-hospital mortality was zero.One patient of transient postoperative neurologic dysfunction, one of acute renal failure, one of transient limbs disturbance, one of secondary thoracotomy operation, one of gastrointestinal hemorrhage and one of postoperative chylothorax were observed.CT angiography rechecked showed the position of the vascular stent were satisfactory and the blood flow of arterial branches stents were lucid .The false lumen of the aortic arch and descending aorta closed with thrombus or shrinked.Conclusions The patients required aortic arch to be reconstructed which had no main tearing of intima in the arch may be best candidates for this technique.Open triple-branched stent graft placement combined ascending aorta replacement is an effective means for aortic arch reconstruction in acute Stanford type A aortic dissection.

  19. Dilation of the ascending aorta in Turner syndrome - a prospective cardiovascular magnetic resonance study

    Directory of Open Access Journals (Sweden)

    Pedersen Erik M

    2011-04-01

    Full Text Available Abstract Background The risk of aortic dissection is 100-fold increased in Turner syndrome (TS. Unfortunately, risk stratification is inadequate due to a lack of insight into the natural course of the syndrome-associated aortopathy. Therefore, this study aimed to prospectively assess aortic dimensions in TS. Methods Eighty adult TS patients were examined twice with a mean follow-up of 2.4 ± 0.4 years, and 67 healthy age and gender-matched controls were examined once. Aortic dimensions were measured at nine predefined positions using 3D, non-contrast and free-breathing cardiovascular magnetic resonance. Transthoracic echocardiography and 24-hour ambulatory blood pressure were also performed. Results At baseline, aortic diameters (body surface area indexed were larger at all positions in TS. Aortic dilation was more prevalent at all positions excluding the distal transverse aortic arch. Aortic diameter increased in the aortic sinus, at the sinotubular junction and in the mid-ascending aorta with growth rates of 0.1 - 0.4 mm/year. Aortic diameters at all other positions were unchanged. The bicuspid aortic valve conferred higher aortic sinus growth rates (p Conclusion A general aortopathy is present in TS with enlargement of the ascending aorta, which is accelerated in the presence of a bicuspid aortic valve.

  20. Ortner's syndrome: a case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Dutra, Bruno Landim; Campos, Lenilton da Costa; Marques, Helder de Castro; Vilela, Vagner Moyses, E-mail: brunolandim@yahoo.com.br [Universidade Federal de Juiz de Fora (HU/UFJF), MG (Brazil). Hospital Universitario. Unidade de Radiologia e Diagnostico por Imagem; Carvalho, Rodolfo Elias Diniz da Silva [Centro de Diagnostico Medico (CRM), Sao Mateus, ES (Brazil); Duque, Andre Geraldo da Silva [Axial Medicina Diagnostica, Belo Horizonte, MG (Brazil)

    2015-07-15

    The authors report the case of a 55-year-old female, hypertensive, smoker patient presenting with dysphonia, dysphagia and persistent dry cough. Laryngoscopy diagnosed left vocal cord paralysis. Computed tomography demonstrated saccular aneurysm of the inferior wall of the aortic arch, stretching the left recurrent laryngeal nerve, a finding compatible with Ortner's syndrome. (author)

  1. 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

  2. Occlusive ascending aorta and arch atheroma treated with deep hypothermic circulatory arrest and thromboendarterectomy.

    LENUS (Irish Health Repository)

    O' Sullivan, Katie E

    2013-12-01

    We describe an uncommon presentation of severely advanced aortic atherosclerosis in a 48-year old man with a history of hypertension and heavy smoking. Initial presentation with upper limb ischaemia led to the diagnosis of an aortic arch atheroma occluding 90% of the aortic lumen, managed with deep hypothermic circulatory arrest and aortic thromboendarterectomy. To our knowledge, this is the first reported case of atherosclerotic plaque resulting in aortic occlusion and requiring emergent operative intervention.

  3. 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

  4. Missense mutations in FBN1 exons 41 and 42 cause Weill-Marchesani syndrome with thoracic aortic disease and Marfan syndrome.

    Science.gov (United States)

    Cecchi, Alana; Ogawa, Naomi; Martinez, Hugo R; Carlson, Alicia; Fan, Yuxin; Penny, Daniel J; Guo, Dong-chuan; Eisenberg, Steven; Safi, Hazim; Estrera, Anthony; Lewis, Richard A; Meyers, Deborah; Milewicz, Dianna M

    2013-09-01

    Mutations in FBN1 cause a range of overlapping but distinct conditions including Marfan syndrome (MFS), Weill-Marchesani syndrome (WMS), familial thoracic aortic aneurysms/dissections (FTAAD), acromicric dysplasia (AD), and geleophysic dysplasia (GD). Two forms of acromelic dysplasia, AD and GD, characterized by short stature, brachydactyly, reduced joint mobility, and characteristic facies, result from heterozygous missense mutations occurring in exons 41 and 42 of FBN1; missense mutations in these exons have not been reported to cause MFS or other syndromes. Here we report on probands with MFS and WMS who have heterozygous FBN1 missense mutations in exons 41 and 42, respectively. The proband with WMS has ectopia lentis, short stature, thickened pinnae, tight skin, striae atrophicae, reduced extension of the elbows, contractures of the fingers and toes, and brachydactyly and has a missense mutation in exon 42 of FBN1 (c.5242T>C; p.C1748R). He also experienced a previously unreported complication of WMS, an acute thoracic aortic dissection. The second proband displays classic characteristics of MFS, including ectopia lentis, skeletal features, and aortic root dilatation, and has a missense mutation in exon 41 of FBN1 (c.5084G>A; p.C1695Y). These phenotypes provide evidence that missense mutations in exons 41 and 42 of FBN1 lead to MFS and WMS in addition to AD and GD and also suggest that all individuals with pathogenic FBN1 mutations in these exons should be assessed for thoracic aortic disease and ectopia lentis. Further studies are necessary to elucidate the factors responsible for the different phenotypes associated with missense mutations in these exons of FBN1.

  5. Antegrade Versus Retrograde Cerebral Perfusion in Relation to Postoperative Complications Following Aortic Arch Surgery for Acute Aortic Dissection Type A%双侧顺行与上腔逆行脑灌注在A型主动脉夹层中应用效果对比的临床研究

    Institute of Scientific and Technical Information of China (English)

    吴智勇; 王志维; 夏军; 张遵严; 陈雪芬; 邓宏平; 徐鹏; 李罗成; 王杰

    2011-01-01

    Objective Antegrade or retrograde cerebral perfusion ( RCP) , is a well - established method of brain protection in aortic arch surgery. In this retrospective study, we compard the two methods of brain perfusion. Methods From Oct 2008 to Jan 2011,63 consecutive patients were urgently operated for acute type A aortic dissection and underwent arch replacement under deep hypothermic circulatory arrest (DHCA). Our patient cohort was divided into those protected with antegrade cerebral perfusion ( ACP) (group A, n = 54) and those protected with RCP (group B, n = 9). Results There was no siguificant difference in operative time, cardiopulmonary bypass - time, myocardial blocking time, cerebral - perfusion time between groups B and A, but the incidence of temporary neurologic dysfunction, the mean extubation time,the mean ICU -stay,the hospital -stay increased. Conclusion The antegrade perfusion was related with significantly lower incidence of temporary neurological complications, earlier extubation, shorter ICU - stay, and hospitalization.%目的 深低温停循环(DHCA)的同时顺行或逆行脑灌注,是一种行之有效的脑保护方法.在这个回顾性研究中,我们比较顺行、逆行两种方法的临床疗效.方法 从2008年10月~2011年1月,63例患者行Stanford A型主动脉夹层手术,均在深低温停循环下行全弓置换.其中选择双侧顺行脑灌注的A组病人54例,B组采用上腔静脉逆灌(RCP)脑保护的病人9例.结果 B组与A组比较在手术时间、体外循环时间、心肌阻断时间、脑灌注时间差异不明显,而B组短暂脑神经功能障碍发生率、拔管时间、ICU滞留时间、住院时间有所增加.结论 顺行脑灌注较上腔逆灌降低短暂脑神经功能障碍发生率从而拔管早,缩短ICU及住院时间.

  6. Histology of a Marfan aorta 4.5 years after personalized external aortic root support.

    Science.gov (United States)

    Pepper, John; Goddard, Martin; Mohiaddin, Raad; Treasure, Tom

    2015-09-01

    In 2008, a 26-year old man had personalized external aortic root support (PEARS) with a macroporous mesh. He was the 16th of 46 patients to have this operation. He had a typical Marfan habitus. His mother died of this disease as did his brother, with an aortic dissection. The patient himself died suddenly 4.5 years after his PEARS operation. At autopsy, there was no blood in the pericardium. The coronary orifices and proximal arteries were normal. His bicuspid aortic valve was minimally regurgitant as it was prior to operation and remained throughout follow-up. Macroscopically the implanted mesh was embedded in the adventitia and not separable from the aortic wall. Microscopically it was fully incorporated with collagen fibres as has been seen in our animal studies. The unsupported aortic arch showed some focal fragmentation of elastic fibres and a mild increase in mucopolysaccharides consistent with Marfan syndrome. These appearances were not present in the supported aortic root, which had the histological appearance of a normal aorta. He was the first patient to die with an implant. The histological appearances suggest the possibility that the incorporated support of the aortic root allowed recovery of the microstructure of the media.

  7. Hypoplastic left heart syndrome

    Directory of Open Access Journals (Sweden)

    Thiagarajan Ravi

    2007-05-01

    Full Text Available Abstract Hypoplastic left heart syndrome(HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch. Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision

  8. Aortic insufficiency

    Science.gov (United States)

    ... page, please enable JavaScript. Aortic insufficiency is a heart valve disease in which the aortic valve does not close ... aortic insufficiency Images Aortic insufficiency References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil ...

  9. 经右侧桡动脉入路支架成形术治疗牛角型或Ⅲ型主动脉弓合并颈动脉狭窄%Treatment of bovine type and type Ⅲ aortic arch carotid stenosis with stent angioplasty via the right radial approach

    Institute of Scientific and Technical Information of China (English)

    许岗勤; 王子亮; 汪勇锋; 梁晓东; 李立; 李天晓

    2016-01-01

    Objective To investigate the feasibility and safety of the treatment of type Ⅲ aortic arch right carotid stenosis and bovine type aortic arch left carotid stenosis with stent angioplasty via the right radial approach.Methods From September 2015 to February 2016,the clinical data of 16 patients with type Ⅲ aortic arch right carotid stenosis and bovine type aortic arch left carotid stenosis treated with carotid stent angioplasty via the radial approach at the Cerebrovascular Interventional Therapy Center,Henan Provincial People's Hospital were analyzed retrospectively,including operation success rate,cardio-cerebrovascular complications,and radial artery puncture complications.All 16 patients were treated with endovascular treatment via right radial approach.The coaxial technique was used for guiding catheter to the super-select target vessels.From January 2014 to December 2015,20 patients with type Ⅲ aortic arch right carotid stenosis and bovine type aortic arch left carotid stenosis treated with carotid stent operation were selected at the same time.The ray irradiation time between the two groups were compared.The follow-up time was 3 months.Results The surgical success rate of the 16 patients was 100%.Three patients had decreased blood pressure and heart rate during the operation.None of them had radial artery occlusion or severe cardio-cerebrovascular events.The ray irradiation time (9.94 ± 0.97 min) in patients with carotid artery stenosis stenting via the radial approach was less than those via the femoral artery approach(11.51 ± 1.39 min).There was significant difference (P =0.001).Conclusion The success rate of the type Ⅲ aortic arch right carotid and bovine type aortic arch left carotid stenosis with stent angioplasty via the right radial approach is high,safe and feasible.%目的 探讨经右侧桡动脉入路支架成形术治疗牛角型主动脉弓或Ⅲ型主动脉弓合并颈动脉狭窄的可行性及安全性.方法 回顾性分析2015

  10. The Acute aortic syndrome – what do we know and what should we know?

    DEFF Research Database (Denmark)

    Houlind, Kim Christian

    , complicating the distinction between an IMH and a thrombosed false lumen of an AD. In 2000 Shimizu et al(1) published a series of 96 patients admitted on the diagnosis ”Aortic dissection”. On CT evaluation 51 of these turned out to have an IMH without an intimal tear. Interestingly, this subgroup of patients......Aortic dissection (AD)was first described in 1760. Treatment options have been medical and surgical and are guided by the Stanford and De Bakey classifications based on the location of intimal tears and false lumens. More recently, endovascular treatment options have been suggested. Penetrating....... Intramural hematomas (IMH) are often found on CT-scans in patients with typical aortic pain. They are characterized by the presence of a hematoma in the media , but the absence of flow in the false lumen and the absence of a primary intimal tear. An intimal tear may, however, occur secondary to the IMH...

  11. 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.

  12. Jarcho-Levin syndrome: a new case report with unusual unexplained aortic root dilatation.

    Science.gov (United States)

    Galguera, M A; García, F L; Bauzá Rossi, J; Vázquez de Corral, L

    1997-01-01

    Since Jarcho and Levin described a condition involving extensive vertebral malformations and early death in 1938, many cases have been reported using multiple synonyms. Later, Solomon (3) proposed a subtype classification system to improve counseling concerning risk of recurrence, management, and prognosis. This is a report of a new Hispanic case with findings of spondylothoracic dysostosis and unusual aortic root dilatation.

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

    Science.gov (United States)

    Izumi, Manabu; Teraoka, Shoko; Yamashita, Keisuke; Matsumoto, Kenji; Muronoi, Tomohiro; Izawa, Yoshimitsu; Yonekawa, Chikara; Ano, Masaki; Suzukawa, Masayuki

    2011-01-01

    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.

  14. 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.

  15. Correction of aortic coarctation in a girl with severe PHACE syndrome.

    Science.gov (United States)

    Xiong, Lian; Xia, Zhenkun; Fan, Chengming; Zhang, Weizhi; Yang, Jinfu

    2014-10-14

    A 12-year-old Chinese girl was demonstrated multivessel distortion and malformation: aortic coarctation with the narrowest lumen diameter measuring of 4 mm located between the left common carotid artery and the left subclavian artery, a huge and thin-walled aneurysm is connected to the coarctation, and the descending aorta was distorted. Cerebrovascular revealed distorted arteries and a completely aberrant brain blood supply. She underwent correction of the aortic coarctation by establishing a bypass between the ascending aorta and the descending aorta using a 13-mm Gore-tex tube. Postoperative recovery was uneventful, at 6-month follow-up, the cervical vascular pulsatility was relieved and she is in good condition.

  16. TGFB2 loss of function mutations cause familial thoracic aortic aneurysms and acute aortic dissections associated with mild systemic features of the Marfan syndrome

    Science.gov (United States)

    Boileau, Catherine; Guo, Dong-Chuan; Hanna, Nadine; Regalado, Ellen S.; Detaint, Delphine; Gong, Limin; Varret, Mathilde; Prakash, Siddharth; Li, Alexander H.; d’Indy, Hyacintha; Braverman, Alan C.; Grandchamp, Bernard; Kwartler, Callie S.; Gouya, Laurent; Santos-Cortez, Regie Lyn P.; Abifadel, Marianne; Leal, Suzanne M.; Muti, Christine; Shendure, Jay; Gross, Marie-Sylvie; Rieder, Mark J.; Vahanian, Alec; Nickerson, Deborah A.; Michel, Jean Baptiste; Jondeau, Guillaume; Milewicz, Dianna M.

    2014-01-01

    A predisposition for thoracic aortic aneurysms leading to acute aortic dissections can be inherited in families in an autosomal dominant manner. Genome-wide linkage analysis of two large unrelated families with thoracic aortic disease, followed by whole exome sequencing of affected relatives, identified causative mutations in TGFB2. These mutations, a frameshift mutation in exon 6 and a nonsense mutation in exon 4, segregated with disease with a combined LOD score of 7.7. Sanger sequencing of 276 probands from families with inherited thoracic aortic disease identified two additional TGFB2 mutations. TGFB2 encodes the transforming growth factor beta-2 (TGF-β2) and the mutations are predicted to cause haploinsufficiency for TGFB2, but aortic tissue from cases paradoxically shows increased TGF-β2 expression and immunostaining. Thus, haploinsufficiency of TGFB2 predisposes to thoracic aortic disease, suggesting the initial pathway driving disease is decreased cellular TGF-β2 levels leading to a secondary increase in TGF-β2 production in the diseased aorta. PMID:22772371

  17. Debranching Solutions in Endografting for Complex Thoracic Aortic Dissections

    Energy Technology Data Exchange (ETDEWEB)

    Goksel, Onur Selcuk, E-mail: onurgokseljet@gmail.com [Istanbul University, Istanbul Medical Faculty, Cardiovascular Surgery, Istanbul (Turkey); Guven, Koray [Istanbul University, Istanbul Medical Faculty, Radiology, Istanbul (Turkey); Karatepe, Celalettin [Mustafa Kemal Medical Faculty, Cardiovascular Surgery, Istanbul (Turkey); Gok, Emre [Istanbul University, Istanbul Medical Faculty, Cardiovascular Surgery, Istanbul (Turkey); Acunas, Bulent [Istanbul University, Istanbul Medical Faculty, Radiology, Istanbul (Turkey); Cinar, Bayer [Medical Park Hospital, Istanbul (Turkey); Alpagut, Ufuk [Istanbul University, Istanbul Medical Faculty, Cardiovascular Surgery, Istanbul (Turkey)

    2014-08-15

    Conventional surgical repair of thoracic aortic dissections is a challenge due to mortality and morbidity risks. We analyzed our experience in hybrid aortic arch repair for complex dissections of the aortic arch. Between 2009 and 2013, 18 patients (the mean age of 67 ± 8 years-old) underwent hybrid aortic arch repair. The procedural strategy was determined on the individual patient. Thirteen patients had type I repair using trifurcation and another patient with bifurcation graft. Two patients had type II repair with replacement of the ascending aorta. Two patients received extra-anatomic bypass grafting to left carotid artery allowing covering of zone 1. Stent graft deployment rate was 100%. No patients experienced stroke. One patient with total debranching of the aortic arch following an acute dissection of the proximal arch expired 3 months after TEVAR due to heart failure. There were no early to midterm endoleaks. The median follow-up was 20 ± 8 months with patency rate of 100%. Various debranching solutions for different complex scenarios of the aortic arch serve as less invasive procedures than conventional open surgery enabling safe and effective treatment of this highly selected subgroup of patients with complex aortic pathologies.

  18. 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.

  19. Surgical Treatment of Patients Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Conditions (GenTAC)

    Science.gov (United States)

    Song, Howard K.; Bavaria, Joseph E.; Kindem, Mark W.; Holmes, Kathryn W.; Milewicz, Dianna M.; Maslen, Cheryl L.; Pyeritz, Reed E.; Basson, Craig T.; Eagle, Kim; Tolunay, H. Eser; Kroner, Barbara L.; Dietz, Hal; Menashe, Victor; Devereux, Richard B.; Desvigne-Nickens, Patrice; Ravekes, William; Weinsaft, Jonathan W.; Brambilla, Donald; Stylianou, Mario P.; Hendershot, Tabitha; Mitchell, Megan S.; LeMaire, Scott A.

    2011-01-01

    Background Genetic disorders are an important cause of thoracic aortic aneurysms (TAAs) in young patients. Despite advances in the treatment of genetically triggered TAAs, the optimal syndrome-specific treatment approach remains undefined. We used data from the NIH-funded, multicenter National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) to characterize the contemporary surgical treatment of patients with genetically triggered TAAs. Methods GenTAC’s aim is to collect longitudinal clinical data and banked biospecimens from 2800 patients with genetically triggered TAAs. We analyzed data from all patients enrolled in GenTAC to date whose clinical data were available (n=606; mean age, 37.5 years). Results The patients’ primary diagnoses included Marfan syndrome (35.8%), bicuspid aortic valve with aneurysm (29.2%), and familial TAAs and dissections (10.7%). More than half of patients (56.4%) had undergone at least 1 operation; the most common indications were aneurysm (85.7%), valve dysfunction (65.8%), and dissection (25.4%). Surgical procedures included replacement of the aortic root (50.6%), ascending aorta (64.8%), aortic arch (27.9%), and descending or thoracoabdominal aorta (12.4%). Syndrome-specific differences in age, indications for surgery, and procedure type were identified. Conclusions Patients with genetically transmitted TAAs evaluated in tertiary care centers frequently undergo surgery. Aneurysm repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like TAAs themselves, complications of TAAs, including dissection and aortic valve dysfunction, are important indications for surgery. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of patients with TAAs. PMID:19699898

  20. Aortic dissection

    Science.gov (United States)

    ... linked to aortic dissection include: Bicuspid aortic valve Coarctation (narrowing) of the aorta Connective tissue disorders (such ... failure Aneurysm Aortic insufficiency Blood clots Cardiac tamponade Coarctation of the aorta Hardening of the arteries High ...

  1. Further insights into the syndrome of prolapsing non-coronary aortic cusp and ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Akhlaque N Bhat

    2012-03-01

    Full Text Available Ventricular septal defect ( VSD with prolapse of the right coronary cusp and aortic regurgitation can be managed surgically with the anatomical correction technique . However when the VSD is located underneath the non coronary cusp surgical management differs due to anatomical constraints and secondary pathological changes seen in the non coronary cusp. It is therefore important that the location of the VSD and the morphology of prolapsing cusp be characterised preoperatively in order to plan appropriate surgical repair. We present a case study in which we discuss the salient differences in the surgical management of the prolapsing right and the prolapsing non coronary cusps.

  2. [Paradigm shifts in aortic pathology: clinical and therapeutic implications. Clinical imaging in chronic and acute aortic syndromes. The aorta as a cause of cardiac disease].

    Science.gov (United States)

    Nistri, Stefano; Roghi, Alberto; Mele, Donato; Biagini, Elena; Chiodi, Elisabetta; Colombo, Ettore; d'Amati, Giulia; Leone, Ornella; Angelini, Annalisa; Basso, Cristina; Pepe, Guglielmina; Rapezzi, Claudio; Thiene, Gaetano

    2014-06-01

    Multimodal imaging plays a pivotal role in the assessment of the thoracic aorta, both in chronic and acute settings. Moving from improved knowledge on the structure and function of the aortic wall, as well as on its pathophysiology and histopathology, appropriate utilization of each imaging modality results into a better definition of the patient's need and proper treatment strategy. This review is aimed at highlighting the most critical aspects in this field, providing cardiologists with some novel clues for the imaging approach to patients with thoracic aortic disease.

  3. Castration modifies aortic vasoreactivity and serum fatty acids in a sucrose-fed rat model of metabolic syndrome.

    Science.gov (United States)

    Perez, Israel; El Hafidi, Mohammed; Carvajal, Karla; Baños, Guadalupe

    2009-03-01

    Levels of testosterone and estradiol influence the incidence of cardiovascular diseases: generally, estrogens in females are protective before menopause; coronaropathies, hypertension, and dyslipidemias in normal men are more frequent at comparable ages. We investigated the modulation by castration of in vitro vasoreactivity, serum lipid content, and systolic blood pressure (SBP) in rats with sucrose-induced metabolic syndrome. The main characteristics of the rat model are: hypertriglyceridemia, moderately high blood pressure, intra-abdominal accumulation of adipose tissue, hyperinsulinemia, nephropathy, increased oxidative stress, and altered vasoreactivity. Male weanling rats received 30% sucrose solution for 16 weeks (metabolic syndrome; MS), controls (C) had plain water; both had commercial rodent chow. They were subdivided into five groups with two subgroups each: Group 1, intact C and MS rats, Groups 2-5, C and MS rats castrated for periods of 16, 12, 8, and 4 weeks. At the end of the study period, systolic blood pressure was measured, and blood and aortas were obtained for fatty acid determination and vasoreactivity assays, respectively. After 16 weeks' sucrose treatment MS aortas showed hypercontractility and decreased vasodilation. Palmitic and palmitoleic acids were increased in MS versus C. Arachidonic acid levels in MS were lower than in intact or castrated C. Long-term castration of 16 weeks normalized the levels of palmitic and oleic acids. With the shorter periods of castration, contractility increased and relaxation decreased in C and MS, but it was more significant in C. Regarding fatty acid composition, long-term castration increased polyunsaturated (arachidonic and eicosapentaenoic) fatty acids. The shorter periods did not modify the fatty acid profile in either C or MS. Metabolic syndrome altered SBP, aortic reactivity, and levels of fatty acids; castration of long duration normalized them in some cases.

  4. Effects of aortic irregularities on blood flow.

    Science.gov (United States)

    Prahl Wittberg, Lisa; van Wyk, Stevin; Fuchs, Laszlo; Gutmark, Ephraim; Backeljauw, Philippe; Gutmark-Little, Iris

    2016-04-01

    Anatomic aortic anomalies are seen in many medical conditions and are known to cause disturbances in blood flow. Turner syndrome (TS) is a genetic disorder occurring only in females where cardiovascular anomalies, particularly of the aorta, are frequently encountered. In this study, numerical simulations are applied to investigate the flow characteristics in four TS patient- related aortic arches (a normal geometry, dilatation, coarctation and elongation of the transverse aorta). The Quemada viscosity model was applied to account for the non-Newtonian behavior of blood. The blood is treated as a mixture consisting of water and red blood cells (RBC) where the RBCs are modeled as a convected scalar. The results show clear geometry effects where the flow structures and RBC distribution are significantly different between the aortas. Transitional flow is observed as a jet is formed due to a constriction in the descending aorta for the coarctation case. RBC dilution is found to vary between the aortas, influencing the WSS. Moreover, the local variations in RBC volume fraction may induce large viscosity variations, stressing the importance of accounting for the non-Newtonian effects.

  5. Modificação técnica para correção de coarctação aórtica com hipoplasia do arco aórtico Technical modification for correction of aortic coarctation using hypoplastic arch

    Directory of Open Access Journals (Sweden)

    José Alberto Caliani

    2008-09-01

    2005 and July 2006, nine newborn patients with aortic coarctation and significant aortic hypoplasia were selected, and underwent a new surgical approach in order to correct this aortic defect. The definition of aortic arch hypoplasia according to Moulaert's criteria is an aortic arch with a diameter that is less than 50% of the diameter of the ascending aorta. In this study, only patients with proximal and distal aortic hypoplasia were selected. Many techniques were previously used, but significant residual gradients were observed, as well as the incovenience of definitive occlusion of the left subclavian artery. The aim of this study is to describe a new surgical technique that includes left posterolateral thoracotomy, wide mobilization of descending aorta with occlusion of the first two intercostal branches, transection of the left subclavian artery at its base, wide resection of the hypoplastic area and the surronding regions of the ductus arteriosus; end-to-end anastomosis between the aortic arch and descending aorta, with 7-0 PDS thread and reimplantation of the subclavian artery into the left carotid artery with side-to-end anastomosis. RESULTS: There were no perioperative or late deaths. The mean residual gradient was 5 mmHg. Up to now, there were no cases of recoarctation or medullary neurological lesions. CONCLUSION: Despite the small number of cases and the short follow-up, this technique modification may be an excellent option for the treatment of this complex situation.

  6. Nitric oxide mediates aortic disease in mice deficient in the metalloprotease Adamts1 and in a mouse model of Marfan syndrome.

    Science.gov (United States)

    Oller, Jorge; Méndez-Barbero, Nerea; Ruiz, E Josue; Villahoz, Silvia; Renard, Marjolijn; Canelas, Lizet I; Briones, Ana M; Alberca, Rut; Lozano-Vidal, Noelia; Hurlé, María A; Milewicz, Dianna; Evangelista, Arturo; Salaices, Mercedes; Nistal, J Francisco; Jiménez-Borreguero, Luis Jesús; De Backer, Julie; Campanero, Miguel R; Redondo, Juan Miguel

    2017-02-01

    Heritable thoracic aortic aneurysms and dissections (TAAD), including Marfan syndrome (MFS), currently lack a cure, and causative mutations have been identified for only a fraction of affected families. Here we identify the metalloproteinase ADAMTS1 and inducible nitric oxide synthase (NOS2) as therapeutic targets in individuals with TAAD. We show that Adamts1 is a major mediator of vascular homeostasis, given that genetic haploinsufficiency of Adamts1 in mice causes TAAD similar to MFS. Aortic nitric oxide and Nos2 levels were higher in Adamts1-deficient mice and in a mouse model of MFS (hereafter referred to as MFS mice), and Nos2 inactivation protected both types of mice from aortic pathology. Pharmacological inhibition of Nos2 rapidly reversed aortic dilation and medial degeneration in young Adamts1-deficient mice and in young or old MFS mice. Patients with MFS showed elevated NOS2 and decreased ADAMTS1 protein levels in the aorta. These findings uncover a possible causative role for the ADAMTS1-NOS2 axis in human TAAD and warrant evaluation of NOS2 inhibitors for therapy.

  7. Association of Aortic Calcification on Plain Chest Radiography with Obstructive Coronary Artery Disease

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Yeong Han; Chang, Jeong Ho [Dept. of Diagnostic Radiology, Daegu Catholic University Hospital, Daegu (Korea, Republic of); Park, Jong Sam [Dept. of Radiologic Tecnology, Daegu Health College, Daegu (Korea, Republic of)

    2009-03-15

    This study was conducted to determine an association between aortic calcification viewed on plain chest radiography and obstructive coronary artery disease. Retrospective review of all chest radiography obtained from consecutive patients undergoing coronary angiography. Chest PA images were reviewed by technical radiologist and radiologist. Considering the presence of aortic arch calcification, images were compared with the results of coronary angiography. In addition, the size of aortic arch calcification were divided into two groups - the smaller and the larger than 10 mm. Among the total 846 patients, the number of the patients with obstructive coronary artery disease is total 417 (88.3%) in males and 312 (83.4%) in females. Considering the presence of aortic arch calcification, the positive predictive value of relation between aortic arch calcification and obstructive coronary artery disease was 91.4% and the relative risk of the group with aortic arch calcification to the opposite group was 1.10. According to the size of aortic arch calcification and obstructive coronary artery disease, the positive predictive value was 91.9% and the relative risk between two groups was 1.04. This study shows that aortic calcification was closely associated with obstructive coronary artery disease. If the aortic calcification is notified on plain chest radiography, we strongly recommend to consult with doctor.

  8. Hutchinson–Gilford progeria syndrome with severe calcific aortic valve stenosis

    Science.gov (United States)

    Hanumanthappa, Natesh B; Madhusudan, Ganigara; Mahimarangaiah, Jayaranganath; Manjunath, Cholenahally N

    2011-01-01

    Hutchinson–Gilford progeria syndrome (HGPS) is a rare premature aging syndrome that results from mutation in the Laminin A gene. This case report of a 12-year-old girl with HGPS is presented for the rarity of the syndrome and the classical clinical features that were observed in the patient. All patients with this condition should undergo early and periodic evaluation for cardiovascular diseases. However, the prognosis is poor and management is mainly conservative. There is no proven therapy available. Mortality in this uniformly fatal condition is primarily due to myocardial infarction, strokes or congestive cardiac failure between ages 7 and 21 years due to the rapidly progressive arteriosclerosis involving the large vessels. PMID:21976890

  9. Hutchinson-Gilford progeria syndrome with severe calcific aortic valve stenosis

    Directory of Open Access Journals (Sweden)

    Natesh B Hanumanthappa

    2011-01-01

    Full Text Available Hutchinson-Gilford progeria syndrome (HGPS is a rare premature aging syndrome that results from mutation in the Laminin A gene. This case report of a 12-year-old girl with HGPS is presented for the rarity of the syndrome and the classical clinical features that were observed in the patient. All patients with this condition should undergo early and periodic evaluation for cardiovascular diseases. However, the prognosis is poor and management is mainly conservative. There is no proven therapy available. Mortality in this uniformly fatal condition is primarily due to myocardial infarction, strokes or congestive cardiac failure between ages 7 and 21 years due to the rapidly progressive arteriosclerosis involving the large vessels.

  10. Uremia modulates the phenotype of aortic smooth muscle cells

    DEFF Research Database (Denmark)

    Madsen, Marie; Pedersen, Annemarie Aarup; Albinsson, Sebastian

    2017-01-01

    of moderate uremia in ApoE(-/-) mice increased atherosclerosis in the aortic arch en face 1.6 fold (p = 0.04) and induced systemic inflammation. Based on histological analyses of aortic root sections, uremia increased the medial area, while there was no difference in the content of elastic fibers or collagen...

  11. [Minimally invasive cardiac surgery for aortic valve disease].

    Science.gov (United States)

    Fujimura, Y; Katoh, T; Hamano, K; Gohra, H; Tsuboi, H; Esato, K

    1998-12-01

    Recent surgical advances leading to good operative results have contributed to the trend to useminimally invasive approaches, even in cardiac surgery. Smaller incisions are clearly more cosmetically acceptable to patients. When using a minimally invasive approach, it is most important to maintain surgical quality without jeopardizing patients. A good operative visual field leads to good surgical results. In the parasternal approach, we use a retractor to harvest an internal thoracic artery in coronary artery bypass surgery. Retracting the sternum upward allows for a good surgical view and permits the use of an arch cannula rather than femoral cannulation. When reoperating for aortic valve repair, the j-sternotomy approach requires less adhesiolysis compared with the traditional full sternotomy. No special technique is necessary to perform aortic valve surgery using the j-sternotomy approach. However, meticulous attention must be paid to avoiding left ventricular air embolisms to prevent postoperative stroke or neurocognitive deficits, especially when utilizing a minimally invasive approach. Transesophageal echo is useful not only for monitoring cardiac function but also for monitoring the persence of air in the left ventricle and atrium. This paper compare as the degree of invasion of minimally invasive cardiac surgery and the traditional full sternotomy. No differences were found in the occurrence of systemic inflammatory response syndrome between patients undergoing minimally invasive cardiac surgery and the traditional technique. Therefore it is concluded that minimally invasive surgery for patients with aortic valve disease may become the standard approach in the near future.

  12. Ultrastructural pathology of aortic dissections in patients with Marfan syndrome: Comparison with dissections in patients without Marfan syndrome

    NARCIS (Netherlands)

    K.P. Dingemans; P. Teeling; A.C. van der Wal; A.E. Becker

    2006-01-01

    Despite the discovery in 1990 that mutations in the fibrillin-1 gene cause the Marfan syndrome, the pathogenesis of the life-threatening dissections associated with this disease is far from elucidated. Both the massive number of known fibrillin-1 mutations that result in a heterogeneous patient popu

  13. Treatment of infrarenal abdominal aortic dissection concomitant with an aneurysm

    Institute of Scientific and Technical Information of China (English)

    WANG Li-xin; ZHU Ting; FU Wei-guo; WANG Yu-qi; XI Xun; GUO Da-qiao; CHEN Bin; JIANG Jun-hao; YANG Ju; SHI Zhen-yu

    2007-01-01

    @@ Aortic dissection occurs when layers of the aortic walls are separated by the blood flow through an intimal tear. Dissection of the aorta most frequently originates in the ascending aorta (70%), followed by the descending aorta (22%), the aortic arch (7%) and the abdominal aorta (1/%).1 The dissection limited to the abdominal aorta is rare.2 An isolated abdominal aortic dissection (IAAD) concomitant with an abdominal aortic aneurysm (AAA) is uncommon. We present here one patient with IAAD and AAA treated by endovascular therapy.

  14. CT of thoracic aortic aneurysms.

    Science.gov (United States)

    Posniak, H V; Olson, M C; Demos, T C; Benjoya, R A; Marsan, R E

    1990-09-01

    Aneurysms of the thoracic aorta are most often the result of arteriosclerotic disease. Other causes include degeneration of the medial layer of the aortic wall, either idiopathically or due to genetic disorders such as Marfan syndrome; aortic dissection; trauma; syphilis and other bacterial infection; noninfective aortitis; and congenital anomaly. We review normal anatomy of the aorta and discuss our technique and interpretation of computed tomography (CT) in the evaluation of the thoracic aorta. We illustrate the CT appearance of different types of aortic aneurysms as well as discuss the use of CT for assessing complications of aneurysms, for postoperative follow-up, and in the differentiation of aortic aneurysm from a paraaortic mass.

  15. Severe aortic and arterial aneurysms associated with a TGFBR2 mutation

    Science.gov (United States)

    LeMaire, Scott A; Pannu, Hariyadarshi; Tran-Fadulu, Van; Carter, Stacey A; Coselli, Joseph S; Milewicz, Dianna M

    2008-01-01

    Background A 24-year-old man presented with previously diagnosed Marfan’s syndrome. Since the age of 9 years, he had undergone eight cardiovascular procedures to treat rapidly progressive aneurysms, dissection and tortuous vascular disease involving the aortic root and arch, the thoracoabdominal aorta, and brachiocephalic, vertebral, internal thoracic and superior mesenteric arteries. Throughout this extensive series of cardiovascular surgical repairs, he recovered without stroke, paraplegia or renal impairment. Investigations CT scans, arteriogram, genetic mutation screening of transforming growth factor β receptors 1 and 2. Diagnosis Diffuse and rapidly progressing vascular disease in a patient who met the diagnostic criteria for Marfan’s syndrome, but was later rediagnosed with Loeys–Dietz syndrome. Genetic testing also revealed a de novo mutation in transforming growth factor β receptor 2. Management Regular cardiovascular surveillance for aneurysms and dissections, and aggressive surgical treatment of vascular disease. PMID:17330129

  16. Rarity of isolated pulmonary embolism and acute aortic syndrome occurring outside of the field of view of dedicated coronary CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hwa Yeon; Song, In Sup (Dept. of Diagnostic Radiology Chung-Ang Univ. College of Medicine, Seoul (Korea, Republic of)); Yoo, Seung Min; Rho, Ji Young (Dept. of Diagnostic Radiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)), email: smyoo68@hanmail.net; Moon, Jae Youn; Kim, In Jai; Lim, Sang Wook; Sung, Jung Hoon; Cha, Dong Hun (Dept. of Cardiology CHA Medical Univ. Hospital, Bundang (Korea, Republic of)); White, Charles S. (Dept. of Diagnostic Radiology Univ. of Maryland, Baltimore (United States))

    2011-05-15

    Background Although triple rule-out CT angiography (TRO) to simultaneously evaluate acute coronary syndrome (ACS), pulmonary embolism (PE), and acute aortic syndrome (AAS) is increasingly used in many institutions, TRO is inevitably associated with increased radiation exposure due to extended z-axis coverage compared with dedicated coronary CT angiography (DCTA). Purpose To determine the frequency of exclusion of findings of AAS, PE, and significant incidental non-cardiac pathology that may be the cause of acute chest pain when using a restricted DCTA field of view (FOV). Material and Methods We retrospectively reviewed CT images and charts of 103 patients with acute PE and 50 patients with AAS. Either non-ECG gated dedicated pulmonary or aortic CT angiography was performed using 16- or 64-slice multidetector CT (MDCT). We analyzed the incidence of isolated PE, AAS, or significant non-cardiac pathology outside of DCTA FOV (i.e. from tracheal carina to the base of heart). Results There were two cases of isolated PE (2/103, 1.9%) excluded from the FOV of DCTA. One case of PE was isolated to the subsegmental pulmonary artery in the posterior segment of the right upper lobe. In the second case, pulmonary embolism in the left main pulmonary artery was located out of the FOV of DCTA because the left main pulmonary artery was retracted upwardly by fibrotic scar in the left upper lobe due to prior tuberculosis. There was no case of AAS and significant non-cardiac pathology excluded from the FOV of DCTA. AAS (n = 50) consisted of penetrating atherosclerotic ulcer (n = 7), intramural hematoma (n = 5) and aortic dissection (n = 38). Conclusion As isolated PE, AAS, and significant non-cardiac pathology outside of the DCTA FOV rarely occur, DCTA may replace TRO in the evaluation of patients with non-specific acute chest pain and a low pre-test probability of PE or aortic dissection

  17. Neutron induced teratogenesis and spermatogenesis inhibitor fertilysin induced fetal bis-diamine syndrome in the rat. An animal model for DiGeorge and CATCH22 syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Shoji, Shuneki [Hiroshima Univ., Research Institute for Radiation Biology and Medicine, Hiroshima (Japan)

    2003-07-01

    To develop preventive and regenerative medicine measures and to clarify the effect of neutron-irradiation and Fertilysin on vasculogenesis and teratogenesis, we decided to investigate the pathogenesis of these abnormalities in this study and compare them to abnormalities reported in humans. Pregnant rats were exposed to graded doses of 14.1 MeV neutron irradiation or Fertilysin on day 10 of gestation. The rats were sacrificed on day 18 of gestation, examined for lethality and surviving fetuses, and were microdissected for malformations. Our studies showed that neutron irradiation of rats commonly induced abnormalities whose types included eye, limb and tail defects, transposition of the great arteries, riding aorta, right aortic arch and aortic arch anomalies. These results suggest that maternal exposure to neutron-irradiation may have caused DNA damage and neural crest deficiency in offspring. These results are similar to those found in animal models with Retinoic acid syndrome and human fetuses with DiGeorge syndrome, a condition considered as a pharyngeal arch syndrome related to a cephalic neurocristopathy. In addition, multi-organ malformations associated with the highest incidences of abnormal vasculogenesis, cardiac outflow tracts and aortic arch anomalies such as right aortic arch and aberrant subclavian artery were found to be consistently produced following maternal exposure to Fertilysin on day 10 of gestation. Evidently the crucial scenario for administering Fertilysin to cause the cardiovascular defects of all surviving fetuses, in which over 80% of the fetuses were persistent truncus arteriosus (PTA) and the remainder was tetralogy of Fallot (TOF), is 200 mg for day 10 of gestation. This corresponds in humans to approximately day 21 after conception. A mechanism involving DNA damage, disruption of neural crest cells and growth and transcription factors, as well as growth failure of the branchial arches from apoptosis and neurocristopathy of the third

  18. Turner Syndrome

    Science.gov (United States)

    ... in the inner layer of the aorta (aortic dissection). A defect in the valve between the heart ... Turner syndrome are at increased risk of aortic dissection during pregnancy, they should be evaluated by a ...

  19. Mixed partial anomalous pulmonary venous drainage coexistent with an aortic valve abnormality – analysis of ultrasound diagnostics in a 10-year-old girl with Turner syndrome

    Science.gov (United States)

    Karolczak, Maciej A.; Komarnicka, Justyna; Mirecka, Małgorzata

    2014-01-01

    The authors present a case of echocardiographic diagnosis of a rare congenital cardiovascular anomaly in the form of mixed partial anomalous pulmonary veins connection in a 10-year-old girl with Turner syndrome and congenital mild stenosis of insufficient bicuspid aortic valve, made while diagnosing the causes of intestinal tract bleeding. The article presents various diagnostic difficulties leading to the delayed determination of a correct diagnosis, resulting from the absence of symptoms of circulatory failure in the early stage of the disease and the occurrence of severe and dominant auscultatory phenomena typical for congenital aortic valve defect which effectively masked the syndromes of increased pulmonary flow. The authors discuss the role of the impact of phenotypic characteristics of the Turner syndrome, in particular a short webbed neck restricting the suprasternal echocardiographic access and the presence of psychological factors associated with a long-term illness. The importance of indirect echocardiographic symptoms suggesting partial anomalous pulmonary veins connection in the presence of bicuspid aortic valve, e.g. enlargement of the right atrium and right ventricle, and paradoxical interventricular septum motion were emphasized in patients lacking ASD, pulmonary hypertension or tricupid and pulmonary valve abnormalities. The methodology of echocardiographic examination enabling direct visualization of the abnormal vascular structures was presented. Special attention was paid to the significance of highly sensitive echocardiographic projections: high right and left parasternal views in sagittal and transverse planes with patient lying on the side, with the use of two-dimensional imaging and color Doppler. Finally, the limitations of echocardiography resulting from the visualization and tracking of abnormal vascular structures hidden behind ultrasound non-conductive tissues were indicated, as was the role of other diagnostic modalities, such as angio

  20. Mixed partial anomalous pulmonary venous drainage coexistent with an aortic valve abnormality - analysis of ultrasound diagnostics in a 10-year-old girl with Turner syndrome.

    Science.gov (United States)

    Mądry, Wojciech; Karolczak, Maciej A; Komarnicka, Justyna; Mirecka, Małgorzata

    2014-03-01

    The authors present a case of echocardiographic diagnosis of a rare congenital cardiovascular anomaly in the form of mixed partial anomalous pulmonary veins connection in a 10-year-old girl with Turner syndrome and congenital mild stenosis of insufficient bicuspid aortic valve, made while diagnosing the causes of intestinal tract bleeding. The article presents various diagnostic difficulties leading to the delayed determination of a correct diagnosis, resulting from the absence of symptoms of circulatory failure in the early stage of the disease and the occurrence of severe and dominant auscultatory phenomena typical for congenital aortic valve defect which effectively masked the syndromes of increased pulmonary flow. The authors discuss the role of the impact of phenotypic characteristics of the Turner syndrome, in particular a short webbed neck restricting the suprasternal echocardiographic access and the presence of psychological factors associated with a long-term illness. The importance of indirect echocardiographic symptoms suggesting partial anomalous pulmonary veins connection in the presence of bicuspid aortic valve, e.g. enlargement of the right atrium and right ventricle, and paradoxical interventricular septum motion were emphasized in patients lacking ASD, pulmonary hypertension or tricupid and pulmonary valve abnormalities. The methodology of echocardiographic examination enabling direct visualization of the abnormal vascular structures was presented. Special attention was paid to the significance of highly sensitive echocardiographic projections: high right and left parasternal views in sagittal and transverse planes with patient lying on the side, with the use of two-dimensional imaging and color Doppler. Finally, the limitations of echocardiography resulting from the visualization and tracking of abnormal vascular structures hidden behind ultrasound non-conductive tissues were indicated, as was the role of other diagnostic modalities, such as angio

  1. National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

    Science.gov (United States)

    2016-12-19

    Marfan Syndrome; Turner Syndrome; Ehlers-Danlos Syndrome; Loeys-Dietz Syndrome; FBN1, TGFBR1, TGFBR2, ACTA2 or MYH11 Genetic Mutation; Bicuspid Aortic Valve Without Known Family History; Bicuspid Aortic Valve With Family History; Bicuspid Aortic Valve With Coarctation; Familial Thoracic Aortic Aneurysm and Dissections; Shprintzen-Goldberg Syndrome; Other Aneur/Diss of Thoracic Aorta Not Due to Trauma, <50yo; Other Congenital Heart Disease

  2. A case of abdominal aortic thrombosis associated with the nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Jannet Labidi

    2017-01-01

    Full Text Available Thromboembolic disease is an important and frequent complication in patients with the nephrotic syndrome (NS, and the consequences are often severe. Usually, the venous system is affected. Arterial thrombosis has rarely been reported and occurs mainly in children. We report the case of a 27-year-old man with a history of NS due to focal and segmental glomerulosclerosis resistant to steroids and cyclosporine, admitted for bilateral pain in the calves. Aortogram revealed a suspended thrombus in the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery. Endarterectomy was performed followed by systemic heparinization with a good outcome. Arterial thrombosis is rare and must be prevented.

  3. Coffin-Siris syndrome with the rarest constellation of congenital cardiac defects: A case report with review of literature

    Directory of Open Access Journals (Sweden)

    Lalita Nemani

    2014-01-01

    Full Text Available We report a case of type-A Coffin-Siris syndrome (CSS with a unique constellation of congenital heart defects. A 17-year-old Indian boy was referred to our hospital for central cyanosis with features of right heart failure. The cardiac abnormalities included biventricular outflow tract obstruction, small atrial septal defect (ASD, subaortic ventricular septal defect, drainage of left superior venacava to left atrial appendage, and aortic arch anomaly. Patient underwent successful right ventricular infundibular resection, subaortic membrane resection, closure of atrial and ventricular septal defect, rerouting left superior vena cava to left pulmonary artery and aortic valve replacement.

  4. Intraoral gothic arch tracing.

    Science.gov (United States)

    Rubel, Barry; Hill, Edward E

    2011-01-01

    In order to create optimum esthetics, function and phonetics in complete denture fabrication, it is necessary to record accurate maxillo-mandibular determinants of occlusion. This requires clinical skill to establish an accurate, verifiable and reproducible vertical dimension of occlusion (VDO) and centric relation (CR). Correct vertical relation depends upon a consideration of several factors, including muscle tone, inter-dental arch space and parallelism of the ridges. Any errors made while taking maxillo-mandibular jaw relation records will result in dentures that are uncomfortable and, possibly, unwearable. The application of a tracing mechanism such as the Gothic arch tracer (a central bearing device) is a demonstrable method of determining centric relation. Intraoral Gothic arch tracers provide the advantage of capturing VDO and CR in an easy-to-use technique for practitioners. Intraoral tracing (Gothic arch tracing) is a preferred method of obtaining consistent positions of the mandible in motion (retrusive, protrusive and lateral) at a comfortable VDO.

  5. IL‐6 Regulates Extracellular Matrix Remodeling Associated With Aortic Dilation in a Fibrillin‐1 Hypomorphic mgR/mgR Mouse Model of Severe Marfan Syndrome

    Science.gov (United States)

    Ju, Xiaoxi; Ijaz, Talha; Sun, Hong; LeJeune, Wanda; Vargas, Gracie; Shilagard, Tuya; Recinos, Adrian; Milewicz, Dianna M.; Brasier, Allan R.; Tilton, Ronald G.

    2014-01-01

    Background Development of thoracic aortic aneurysms is the most significant clinical phenotype in patients with Marfan syndrome. An inflammatory response has been described in advanced stages of the disease. Because the hallmark of vascular inflammation is local interleukin‐6 (IL‐6) secretion, we explored the role of this proinflammatory cytokine in the formation of aortic aneurysms and rupture in hypomorphic fibrillin‐deficient mice (mgR/mgR). Methods and Results MgR/mgR mice developed ascending aortic aneurysms with significant dilation of the ascending aorta by 12 weeks (2.7±0.1 and 1.3±0.1 for mgR/mgR versus wild‐type mice, respectively; P<0.001). IL‐6 signaling was increased in mgR/mgR aortas measured by increases in IL‐6 and SOCS3 mRNA transcripts (P<0.05) and in cytokine secretion of IL‐6, MCP‐1, and GM‐CSF (P<0.05). To investigate the role of IL‐6 signaling, we generated mgR homozygous mice with IL‐6 deficiency (DKO). The extracellular matrix of mgR/mgR mice showed significant disruption of elastin and the presence of dysregulated collagen deposition in the medial‐adventitial border by second harmonic generation multiphoton autofluorescence microscopy. DKO mice exhibited less elastin and collagen degeneration than mgR/mgR mice, which was associated with decreased activity of matrix metalloproteinase‐9 and had significantly reduced aortic dilation (1.0±0.1 versus 1.6±0.2 mm change from baseline, DKO versus mgR/mgR, P<0.05) that did not affect rupture and survival. Conclusion Activation of IL‐6‐STAT3 signaling contributes to aneurysmal dilation in mgR/mgR mice through increased MMP‐9 activity, aggravating extracellular matrix degradation. PMID:24449804

  6. Preliminary study on MSCTA technique to determine the shape of aortic arch and its clinical significance%多层螺旋CT血管造影技术判断主动脉弓形态及临床意义的探讨

    Institute of Scientific and Technical Information of China (English)

    王学廷; 董致成; 冯丽; 潘为领

    2014-01-01

    Objective:Using multi-slice spiral CT angiography (MSCTA) technique in evaluating aortic arch form, and to explore the clinical significance.Methods: retrospective analysis of our hospital from 2010 November to 2013 March for cervical and thoracic 256CTA examination in 240 cases without significant vascular disease, no local space occupying disease subjects images, including 147 cases of male, female 93 cases, age 44 ~ 71 years old, the average age is 51.8 years old. Application of CT software respectively measure the internal diameter of carotid artery, brachiocephalic trunk opening to the aortic arch top vertical distance, typing.Results: In 240 patients, 116 cases of type I, 48.33%; II type in 80 cases, accounting for 33.33%; 44 cases of type III, 18.33%. Group statistics according to different age, found that in young group (≤ 45 years) a total of 32 cases, including 24 cases of type I, 75% type II, 8 cases, accounting for 25%, type III in 0 cases, accounting for 0%; the middle-aged group (46 ~ 59 years old) in 104 cases, including 60 cases of type I, 57.69%, II type in 40 cases, accounting for 38.46%, type III in 4 cases, accounting for 3.84%; aged group (≥60 years) in 104 cases, type I in 32 cases, accounting for 30.76%, type II in 32 cases, accounting for 30.76%, 40 cases of type III, 38.46%.Conclusion: MSCTA technology on the morphology of aortic arch judgment accurate, relevant data were examined by MSCTA to obtain the aortic arch diseases before interventional therapy and data, will have the vital significance to neural interventional radiology doctors.%目的:利用多层螺旋CT血管造影(MSCTA)技术评价主动脉弓形态,并探讨其临床意义。方法:回顾性分析行颈胸部联合256CTA检查的240例无重要血管性疾病、无局部占位性疾病的受检者图像,其中男性147例、女性93例,年龄44岁~71岁,平均年龄51.8岁。应用CT测量软件分别测量颈总动脉内径、头臂干开口至主动脉

  7. Unexpected death caused by rupture of a dilated aorta in an adult male with aortic coarctation.

    Science.gov (United States)

    Leth, Peter Mygind; Knudsen, Peter Thiis

    2015-09-01

    Aortic coarctation (AC) is a congenital aortic narrowing. We describe for the first time the findings obtained by unenhanced post mortem computed tomography (PMCT) in a case where the death was caused by cardiac tamponade from a ruptured aneurysmal dilatation of the ascending aorta and the aortic arch without dissection combined with aortic coarctation. The patient, a 46-year-old man, was found dead at home. PMCT showed haemopericardium and dilatation of the ascending aorta and the aortic arch. This appearance led to the mistaken interpretation that the images represented a dissecting aneurysm. The autopsy showed instead a thin-walled and floppy dilatation of the ascending aorta and aortic arch with a coarctation just proximal to the ligamentum arteriosum. A longitudinal tear was found in the posterior aortic wall just above the valves. Blood in the surrounding soft tissue intersected with a large haematoma (1000ml) in the pericardial sac. Cardiac hypertrophy (556g) was observed in the patient, though no other cardiovascular abnormalities were found. Histological analysis showed cystic medial necrosis of the ascending aortic wall. A ruptured aneurysmal dilatation of the ascending aorta and the aortic arch without aortic dissection associated with AC is an uncommon cause of haemopericardium that has only been described a few times before. The case is discussed in relation to other reported cases and in the context of the present understanding of this condition.

  8. 小儿主动脉弓手术中深低温停循环和局部脑灌注的应用效果%Applied research of deep hypothermic circulatory arrest and regional cerebral perfusion in pediatric aortic arch surgery

    Institute of Scientific and Technical Information of China (English)

    郭铮; 郑景浩; 王伟; 付惟定; 朱德明

    2014-01-01

    目的 观察并比较深低温停循环(DHCA)和局部脑灌注(RCP)在小儿主动脉弓手术中的应用效果.方法 70例小于3个月的主动脉缩窄和主动脉弓中断合并房间隔缺损、室间隔缺损的手术患儿根据体外循环方法不同分成DHCA组和RCP组.DHCA组35例,男23例,女12例;年龄(1.33±0.80)个月;体质量(4.3±2.9)kg.RCP组35例,男20例,女15例;年龄(1.35±0.90)个月;体质量(4.4±3.2) kg.分析两组体外循环时间、主动脉阻断时间、停循环或脑灌注时间,呼吸机辅助时间、监护室停留时间,术后肾功能不全、低心排血量综合征、肺部炎症、神经系统并发症和院内死亡等情况.结果 神经系统并发症发病率,DHCA组显著高于RCP组(P<0.05).体外循环时间,RCP组显著的长于DHCA组(P<0.05).RCP时间显著长于DHCA时间(P<0.01).在主动脉阻断时间、呼吸机插管时间、监护室停留时间和术后肾功能不全、低心排血量综合征、肺部炎症及院内死亡比例等两组间差异无统计学意义.结论 RCP是一种有效的脑保护技术,同DHCA相比,能更好的对大脑进行持续的脑血流灌注,适用于复杂耗时的小儿主动脉弓手术,在神经系统保护方面较DHCA有着更好的效果.%Objective To observe and evaluate the effects of the deep hypothermic circulatory arrest(DHCA) and regional cerebral perfusion(RCP) in pediatric aortic arch surgery.Methods According to different methods of CPB,70 infants less than 3-month-old with CoA or IAA were undergone corrective surgery with DHCA or RCP.The bypass time,aortic clamp time,DHCA or RCP time,ventilation time,ICU stay time and post-operative complications were recorded and compared between two groups.Results The incidence of neurological complications was significantly higher in DHCA group.The CPB time was significantly longer in the RCP group,and the RCP time was significantly longer than DHCA time.Blocking time,ventilator intubation time

  9. Imaging of acute aortic diseases; L'imagerie de la pathologie aortique aigue

    Energy Technology Data Exchange (ETDEWEB)

    Semlali, S.; Ennafae, I.; Mahi, M.; Benaissa, L.; Hanine, A.; Akjouj, S.; Jidal, M.; Chaouir, S. [Service d' imagerie medicale, hopital militaire Mohamed V, CHU, Rabat (Morocco)

    2010-09-15

    We report a review of computed tomography (CT) and magnetic resonance (MR) imaging findings in acute aortic syndrome. Contrast-enhanced multidetector CT is a highly accurate imaging method for determining the cause of acute aortic syndrome. (authors)

  10. [Acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult - 2014 AS SMC Guidelines on the classification and diagnosis of aortic diseases].

    Science.gov (United States)

    Gavorník, Peter; Dukát, Andrej; Gašpar, Ľudovít

    2015-01-01

    In addition to organovascular arterial ischemic diseases (cardiovascular, vasculovascular, neurovascular, extre-mitovascular, renovascular, genitovascular, bronchopulmovascular, mesenteriovascular, osteoarthromusculovascular, dermovascular, oculovascular, otovascular, stomatovascular etc.), aortic diseases contribute to the wide spectrum of arterial diseases: aortic aneurysms (AA), acute aortic syndromes (AAS) including aortic dissection (AD), intramural haematoma (IMH), penetrating atherosclerotic ulcer (PAU) and traumatic aortic injury (TAI), pseudoaneurysm, aortic rupture, atherosclerosis, vasculitis as well as genetic diseases (e.g. Turner syndrome, Marfan syndrome, Ehlers-Danlos syndrome) and congenital abnormalities including the coarctation of the aorta (CoA). Similarly to other arterial diseases, aortic diseases may be diagnosed after a long period of subclinical development or they may have an acute presentation. Acute aortic syndrome is often the first sign of the disease, which needs rapid diagnosis and decisionmaking to reduce the extremely poor prognosis. Key clinical-etiology-anatomy-patophysiology (CEAP) diagnostic aspects of aortic diseases are discussed in this document (project Vessels).

  11. Exact monitoring of aortic diameters in Marfan patients without gadolinium contrast: intraindividual comparison of 2D SSFP imaging with 3D CE-MRA and echocardiography

    Energy Technology Data Exchange (ETDEWEB)

    Veldhoen, Simon [University Medical Center Wuerzburg, Department of Diagnostic and Interventional Radiology, Bavaria (Germany); University Medical Center Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); Behzadi, Cyrus; Derlin, Thorsten; Henes, Frank Oliver; Adam, Gerhard; Bannas, Peter [University Medical Center Hamburg-Eppendorf, Department of Diagnostic and Interventional Radiology, Hamburg (Germany); Rybczinsky, Meike; Kodolitsch, Yskert von; Sheikhzadeh, Sara [University Medical Center Hamburg-Eppendorf, Department of General and Interventional Cardiology, Hamburg (Germany); Bley, Thorsten Alexander [University Medical Center Wuerzburg, Department of Diagnostic and Interventional Radiology, Bavaria (Germany)

    2014-10-15

    To assess whether ECG-gated non-contrast 2D steady-state free precession (SSFP) imaging allows for exact monitoring of aortic diameters in Marfan syndrome (MFS) patients using non-ECG-gated contrast-enhanced 3D magnetic resonance angiography (CE-MRA) and echocardiography for intraindividual comparison. Non-ECG-gated CE-MRA and ECG-gated non-contrast SSFP at 1.5 T were prospectively performed in 50 patients. Two readers measured aortic diameters on para-sagittal images identically aligned with the aortic arch at the sinuses of Valsalva, sinotubular junction, ascending/descending aorta and aortic arch. Image quality was assessed on a three-point scale. Aortic root diameters acquired by echocardiography were used as reference. Intra- and interobserver variances were smaller for SSFP at the sinuses of Valsalva (p = 0.002; p = 0.002) and sinotubular junction (p = 0.014; p = 0.043). Image quality was better in SSFP than in CE-MRA at the sinuses of Valsalva (p < 0.0001), sinotubular junction (p < 0.0001) and ascending aorta (p = 0.02). CE-MRA yielded higher diameters than SSFP at the sinuses of Valsalva (mean bias, 2.5 mm; p < 0.0001), and comparison with echocardiography confirmed a higher bias for CE-MRA (7.2 ± 3.4 mm vs. SSFP, 4.7 ± 2.6 mm). ECG-gated non-contrast 2D SSFP imaging provides superior image quality with higher validity compared to non-ECG-gated contrast-enhanced 3D imaging. Since CE-MRA requires contrast agents with potential adverse effects, non-contrast SSFP imaging is an appropriate alternative for exact and riskless aortic monitoring of MFS patients. (orig.)

  12. Asendan Desendan Aortic Bypass: Atan Kalpte Mediyan Sternotomi Yoluyla Onarim

    Directory of Open Access Journals (Sweden)

    Muhammet Akyuz

    2013-10-01

    Full Text Available We report the case of a 9-month-old patient presenting for redo aortic arch surgery because of recoarctation. In present case, ascending-to-descending aortic bypass via median sternotomy was performed without cardiopulmonary bypass with good result. In spite of the fact that the different surgical and intervention treatment options of aortic coarctation are quite satisfactory, a certain group of patients need reoperation because of recoarctation. The recoarctation repair of the aorta with the extra-anatomic aortic bypass is considered a low-risk procedure with high success rate.

  13. Reoperation on aortic disease in patients with previous aortic valve surgery%主动脉瓣置换术后患者再次主动脉外科干预47例

    Institute of Scientific and Technical Information of China (English)

    张良; 常谦; 孙晓刚; 于存涛; 钱向阳

    2013-01-01

    Objective Retrospectively analyze 47 cases received reoperation with aortic disease after aortic valve replacement to deepen the understanding of aortic valve disease.Methods From January 2003 to June 2012,47 patients with previous aortic valve replacement received aortic root or other aortic operation because of new aortic disease.38 male and 9 female,the interval (6.0 ± 3.8) years. All cases with new aortic disease were diagnosed by cardiac ultrasound and aortic computed tomography.Bentall's procedure were operated on 14 patients,total aortic arch replacement with elephant trunk procedure on 14 patients,aortic root and aortic arch with elephant trunk procedure on 7 patients,ascending aortic replacement on 10patients,total thoracic and abdominal aorta replacement on 2 cases.All patients were followed by clinic interview or telephone.Results Aortic dissection and aneurysmal dilatation were occurred on ascending aorta,each account for 50%,in patients with previous aortic valve replacement because of rheumatic valve disease and bicuspid aortic valve; 3 cases with Marfan syndrome occurred ascending aortic dilatation and 4 cases occurred aortic dissection.Diameter in ascending aorta increased (5.2 + 7.1)mm per year and aortic sinus (3.3 ± 3.1)mm per year.The value of ascending aortic dilatation per year in patients with rheumatic disease was higher than patients with Marfan syndrome(P < 0.05).47 patients were re-operated in fuwai hospital,1 patients died in operating room because aortic dissection seriously involved right coronary artery.7 patients have renal insufficiency after operation and all were cured by hemofiltration; neurological complication occurred in 14 patients including that 7 patients stroked and 7 patients had transient brain dysfunciotn.There were no postoperative spinal cord deficits occurred.All patients were followed up,the mean follow up time were(53.49 +33.79) months.8 cases were died during follow-up and threeyear survival rate was 83

  14. Engineered zinc-finger proteins can compensate genetic haploinsufficiency by transcriptional activation of the wild-type allele: application to Willams-Beuren syndrome and supravalvular aortic stenosis.

    Science.gov (United States)

    Zhang, Pei; Huang, Angela; Morales-Ruiz, Manuel; Starcher, Barry C; Huang, Yan; Sessa, William C; Niklason, Laura E; Giordano, Frank J

    2012-11-01

    Williams-Beuren syndrome (WBS) and supravalvular aortic stenosis (SVAS) are genetic syndromes marked by the propensity to develop severe vascular stenoses. Vascular lesions in both syndromes are caused by haploinsufficiency of the elastin gene. We used these distinct genetic syndromes as models to evaluate the feasibility of using engineered zinc-finger protein transcription factors (ZFPs) to achieve compensatory expression of haploinsufficient genes by inducing augmented expression from the remaining wild-type allele. For complex genes with multiple splice variants, this approach could have distinct advantages over cDNA-based gene replacement strategies. Targeting the elastin gene, we show that transcriptional activation by engineered ZFPs can induce compensatory expression from the wild-type allele in the setting of classic WBS and SVAS genetic mutations, increase elastin expression in wild-type cells, induce expression of the major elastin splice variants, and recapitulate their natural stoichiometry. Further, we establish that transcriptional activation of the mutant allele in SVAS does not overcome nonsense-mediated decay, and thus ZFP-mediated transcriptional activation is not likely to induce production of a mutant protein, a crucial consideration. Finally, we show in bioengineered blood vessels that ZFP-mediated induction of elastin expression is capable of stimulating functional elastogenesis. Haploinsufficiency is a common mechanism of genetic disease. These findings have significant implications for WBS and SVAS, and establish that haploinsufficiency can be overcome by targeted transcriptional activation without inducing protein expression from the mutant allele.

  15. 主动脉弓部手术中单侧大脑灌注血流变化与神经系统并发症关系的分析%Analysis of the relationship between the hemodynamic changes during selective cerebral perfusion and postoperative neurological complications in aortic arch surgery

    Institute of Scientific and Technical Information of China (English)

    阳晟; 任长伟; 许尚栋; 孙立忠

    2012-01-01

    Objective: This study tries to monitor the hemodynamic changes of middle cerebral artery ( MCA) by transcranial Doppler sonography (TCD) in aortic arch surgery. Analysis of relationship between the hemodynamic changes during selective cerebral perfusion (SCP) and postoperative neurological complications. Methods: From September 2010 to January 2011, 23 patients with aortic dissection undergoing aortic arch operation were included in this study. Every patient underwent profound hypothermia (DHCA) and selective cerebral perfusion. Hemodynamic signals of bilateral MCA were monitored via TCD during operation. Data at 6 time-points were selected for analysis. All the patients were divided into four groups by the decline in the proportion of non-perfusion side flow( 30% ) , and all the postoperative neurological complications and early death after surgery were recorded in each group. Results: During the full flow of CPB, blood flow velocity of MCA at left and right side was no differences ( P = 0. 565 ) . During SCP, blood flow velocity of MCA at perfusion side (right side) was similar with blood flow velocity before SCP; and blood flow velocity of MCA at non-perfusion side (left side) was significant decreased compare with blood flow veloci-ly before SCP (P 30% groups (50% ) ,no permanent neurological dysfunction (PND) and early death was observed. Conclusion; SCP in aortic arch operation can meet the blood supply to the brain; During SCP, blood flow velocity of MCA at perfusion side (right side) was similar to the blood flow velocity before SCP; blood flow velocity of MCA at non-perfusion side (left side) was significant decreased compare with blood flow velocity before SCP. Less than 30% drop of blood flow velocity of MCA at non-perfusion side (left side) may be safe for the patients.%目的:通过经颅多普勒超声(TCD)监测主动脉弓部手术中大脑中动脉(MCA)的血流,评估选择性脑灌注(SCP)期间血流变化与术后神经系统

  16. Glossary to ARCH (GARCH)

    DEFF Research Database (Denmark)

    Bollerslev, Tim

    The literature on modeling and forecasting time-varying volatility is ripe with acronyms and abbreviations used to describe the many different parametric models that have been put forth since the original linear ARCH model introduced in the seminal Nobel Prize winning paper by Engle (1982......).  The present paper provides an easy-to-use encyclopedic reference guide to this long list of ARCH acronyms.  In addition to the acronyms associated with specific parametric models, I have also included descriptions of various abbreviations associated with more general statistical procedures and ideas...

  17. Combined surgical and catheter-based treatment of extensive thoracic aortic aneurysm and aortic valve stenosis

    DEFF Research Database (Denmark)

    De Backer, Ole; Lönn, Lars; Søndergaard, Lars

    2015-01-01

    endovascular aneurysm repair (TEVAR) has changed and extended management options in thoracic aorta disease, including in those patients deemed unfit or unsuitable for open surgery. Accordingly, transcatheter aortic valve replacement (TAVR) is increasingly used to treat patients with symptomatic severe aortic...... valve stenosis (AS) who are considered at high risk for surgical aortic valve replacement. In this report, we describe the combined surgical and catheter-based treatment of an extensive TAA and AS. To our knowledge, this is the first report of hybrid TAA repair combined with TAVR.......An extensive thoracic aortic aneurysm (TAA) is a potentially life-threatening condition and remains a technical challenge to surgeons. Over the past decade, repair of aortic arch aneurysms has been accomplished using both hybrid (open and endovascular) and totally endovascular techniques. Thoracic...

  18. How to Perfuse: Concepts of Cerebral Protection during Arch Replacement

    Directory of Open Access Journals (Sweden)

    Andreas Habertheuer

    2015-01-01

    Full Text Available Arch surgery remains undoubtedly among the most technically and strategically challenging endeavors in cardiovascular surgery. Surgical interventions of thoracic aneurysms involving the aortic arch require complete circulatory arrest in deep hypothermia (DHCA or elaborate cerebral perfusion strategies with varying degrees of hypothermia to achieve satisfactory protection of the brain from ischemic insults, that is, unilateral/bilateral antegrade cerebral perfusion (ACP and retrograde cerebral perfusion (RCP. Despite sophisticated and increasingly individualized surgical approaches for complex aortic pathologies, there remains a lack of consensus regarding the optimal method of cerebral protection and circulatory management during the time of arch exclusion. Many recent studies argue in favor of ACP with various degrees of hypothermic arrest during arch reconstruction and its advantages have been widely demonstrated. In fact ACP with more moderate degrees of hypothermia represents a paradigm shift in the cardiac surgery community and is widely adopted as an emergent strategy; however, many centers continue to report good results using other perfusion strategies. Amidst this important discussion we review currently available surgical strategies of cerebral protection management and compare the results of recent European multicenter and single-center data.

  19. How to Perfuse: Concepts of Cerebral Protection during Arch Replacement

    Science.gov (United States)

    Habertheuer, Andreas; Wiedemann, Dominik; Kocher, Alfred; Laufer, Guenther; Vallabhajosyula, Prashanth

    2015-01-01

    Arch surgery remains undoubtedly among the most technically and strategically challenging endeavors in cardiovascular surgery. Surgical interventions of thoracic aneurysms involving the aortic arch require complete circulatory arrest in deep hypothermia (DHCA) or elaborate cerebral perfusion strategies with varying degrees of hypothermia to achieve satisfactory protection of the brain from ischemic insults, that is, unilateral/bilateral antegrade cerebral perfusion (ACP) and retrograde cerebral perfusion (RCP). Despite sophisticated and increasingly individualized surgical approaches for complex aortic pathologies, there remains a lack of consensus regarding the optimal method of cerebral protection and circulatory management during the time of arch exclusion. Many recent studies argue in favor of ACP with various degrees of hypothermic arrest during arch reconstruction and its advantages have been widely demonstrated. In fact ACP with more moderate degrees of hypothermia represents a paradigm shift in the cardiac surgery community and is widely adopted as an emergent strategy; however, many centers continue to report good results using other perfusion strategies. Amidst this important discussion we review currently available surgical strategies of cerebral protection management and compare the results of recent European multicenter and single-center data. PMID:26713319

  20. Clarifying the anatomy of the fifth arch artery

    Directory of Open Access Journals (Sweden)

    Saurabh Kumar Gupta

    2016-01-01

    Full Text Available The artery allegedly forming in the fifth pharyngeal arch has increasingly been implicated as responsible for various vascular malformations in patients with congenitally malformed hearts. Observations from studies on developing embryos, however, have failed to provide support to substantiate several of these inferences such that the very existence of the fifth arch artery remains debatable. To the best of our knowledge, in only a solitary human embryo has a vascular channel been found that truly resembled the artery of the fifth arch. Despite the meager evidence to support its existence, the fifth arch artery has been invoked to explain the morphogenesis of double-barreled aorta, some unusual forms of aortopulmonary communications, and abnormalities of the brachiocephalic arteries. In most of these instances, the interpretations have proved fallible when examined in the light of existing knowledge of cardiac development. In our opinion, there are more plausible alternative explanations for the majority of these descriptions. Double-barreled aorta is more likely to result from retention of the recently identified dorsal collateral channels while abnormalities of brachiocephalic arteries are better explained on the basis of extensive remodeling of aortic arches during fetal development. Some examples of aortopulmonary communications, nonetheless, may well represent persistence of the developing artery of the fifth pharyngeal arch. We here present one such case - a patient with tetralogy of Fallot and pulmonary atresia, in whom the fifth arch artery provided a necessary communication between the ascending aorta and the pulmonary arteries. In this light, we discuss the features we consider to be essential before attaching the tag of "fifth arch artery" to a candidate vascular channel.

  1. Copy-Number Variation of the Glucose Transporter Gene SLC2A3 and Congenital Heart Defects in the 22q11.2 Deletion Syndrome

    OpenAIRE

    Mlynarski, Elisabeth E.; Sheridan, Molly B.; Xie, Michael; Guo, Tingwei; Racedo, Silvia E.; McDonald-McGinn, Donna M.; Gai, Xiaowu; Chow, Eva W.C.; Vorstman, Jacob; Swillen, Ann; Devriendt, Koen; Breckpot, Jeroen; Digilio, Maria Cristina; Marino, Bruno; Dallapiccola, Bruno

    2015-01-01

    The 22q11.2 deletion syndrome (22q11DS; velocardiofacial/DiGeorge syndrome; VCFS/DGS) is the most common microdeletion syndrome and the phenotypic presentation is highly variable. Approximately 65% of individuals with 22q11DS have a congenital heart defect (CHD), mostly of the conotruncal type, and/or an aortic arch defect. The etiology of this phenotypic variability is not currently known. We hypothesized that copy-number variants (CNVs) outside the 22q11.2 deleted region might increase the ...

  2. Goldenhar Syndrome Associated with Extensive Arterial Malformations

    Directory of Open Access Journals (Sweden)

    Renee Frances Modica

    2015-01-01

    Full Text Available Goldenhar Syndrome is characterized by craniofacial, ocular and vertebral defects secondary to abnormal development of the 1st and 2nd branchial arches and vertebrae. Other findings include cardiac and vascular abnormalities. Though these associations are known, the specific anomalies are not well defined. We present a 7-month-old infant with intermittent respiratory distress that did not improve with respiratory interventions. Echocardiogram suggested a double aortic arch. Cardiac CT angiogram confirmed a right arch and aberrant, stenotic left subclavian artery, dilation of the main pulmonary artery, and agenesis of the left thyroid lobe. Repeat echocardiograms were concerning for severely dilated coronary arteries. Given dilation, a rheumatologic workup ensued, only identifying few weakly positive autoantibodies. Further imaging demonstrated narrowing of the aorta below the renal arteries and extending into the common iliac arteries and proximal femoral arteries. Given a physical exam devoid of rheumatologic findings, only weakly positive autoantibodies, normal inflammatory markers, and presence of the coronary artery dilation, the peripheral artery narrowings were not thought to be vasculitic. This case illustrates the need to identify definitive anomalies related to Goldenhar Syndrome. Although this infant’s presentation is rare, recognition of specific vascular findings will help differentiate Goldenhar Syndrome from other disease processes.

  3. Current status of diagnosis and endovascular repair for acute aortic syndrome%急性主动脉综合征诊断及腔内治疗现状

    Institute of Scientific and Technical Information of China (English)

    景在平; 冯翔

    2008-01-01

    @@ 主动脉夹层(aortic dissection, AD)、穿透性动脉硬化性溃疡(penetrating atherosclerotic ulcer, PAU)、壁内血肿(intramural hematomas, IMH) 是一组具有相似临床症状的主动脉病变,近几年来,有人提议用急性主动脉综合征(acute aortic syndrome,AAS)来描述主动脉的这组病理改变.

  4. 食管癌切除行弓上食管胃机械吻合术150例%Use of disposable pipe type stapling aortic arch anastomosis of esophagusand stomach on 150 cases of middle and lower esophageal cancer

    Institute of Scientific and Technical Information of China (English)

    贾伟; 于文江; 岳志; 常王玉

    2012-01-01

    Objective To summarize the clinical experience of using of disposable pipe type stapling aortic arch anastomosis of esophagus and stomach. Methods Retrospective analysis was made of 150 cases of middle and lower esophageal cancer resection, with disposable pipe type of stapling bow mechanical esophagogastric anastomosis. Results A hundred and forty-nine cases achieved successful, however,1 patient failed. There was one case of anastomotic bleeding. Postoperative follow-up was performed on 144 cases,3 deaths,the causes of which were anastomotic fistula,a serious infection in 1 ,and aortic esophageal fistula in 2 cases. There was chylothorax in 1 case,cured by symptomatic treatment. The other patients recovered well. Long-term anastomotic stricture appeared in 5 cases, all expanded by balloon dilator to ease or cure after 7 times. 6 cases lost. Conclusion The use of lower esophageal intercostal posterolateral left chest incision and pipe-type esophagus and stomach stapling arch anastomosis performs with surgical difficulty, but there are fewer complications and improved quality of life of patients after the stomach intestine reconstruction.%目的 探讨一次性弯管型吻合器行主动脉弓上食管胃吻合术的临床经验.方法 回顾性分析150例采用一次性弯管型吻合器行弓上食管胃机械吻合根治性切除术中、下段食管癌.结果 一次吻合成功149例,1例失败改为弓旁手工吻合.全组患者1例吻合口出血.术后随访144例,死亡3例,死亡原因:吻合口瘘、严重感染1例,食管主动脉瘘2例.,乳糜胸1例,经对症处理治愈;其余患者术后恢复良好.远期出现吻合口狭窄5例,均经球囊扩张器扩张7次后缓解或治愈.6例失访.结论 中、下段食管癌采用左胸后外侧肋间切口,使用弯管型吻合器行食管胃弓上吻合,手术难度虽较大,但术后胃肠道重建并发症较少,患者的生活质量得到提高.

  5. Outcomes of single-stage total arch replacement via clamshell incision

    Directory of Open Access Journals (Sweden)

    Ishizaka Toru

    2011-09-01

    Full Text Available Abstract Background Treatment of complex aortic pathologies involving the transverse arch with extensive involvement of the descending aorta remains a surgical challenge. Since clamshell incision provides superior exposure of the entire thoracic aorta, we evaluated the use of this technique for single-stage total arch replacement by arch vessel reconstruction. Methods The arch-first technique combined with clamshell incision was used in 38 cases of aneurysm and aortic disease in 2008 and 2009. Extensive total arch replacement was used with clamshell incision for reconstruction of arch vessels under deep hypothermic circulatory arrest. Results Overall 30-day mortality was 13%. The mean operating time was approximately 8 hours. Deep hypothermia resulted in mean CPB time exceeding 4.5 hours and mean duration of circulatory arrest was 25 minutes. The overall postoperative temporary and permanent neurologic dysfunction rates were 3% and 3% for elective and 3% and 0% for emergency surgery, respectively. All patients except the five who died in hospital were discharged without nursing care after an average post-operative hospital stay of 35 days. Conclusions The arch-first technique, combined with clamshell incision, provides expeditious replacement of the thoracic aorta with an acceptable duration of hypothermic circulatory arrest and minimizes the risk of retrograde atheroembolism by using antegrade perfusion.

  6. Aortic stenosis

    Science.gov (United States)

    ... Images Aortic stenosis Heart valves References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil ... ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/ ...

  7. Endovascular repair for abdominal aortic aneurysm followed by type B dissection.

    Science.gov (United States)

    Shingaki, Masami; Kato, Masaaki; Motoki, Manabu; Kubo, Yoji; Isaji, Toshihiko; Okubo, Nobukazu

    2016-10-01

    An 86-year-old man with an abdominal aortic aneurysm was diagnosed with type B aortic dissection accompanied by a patent false lumen that started at the distal arch of the thoracic aorta and terminated at the left common iliac artery. Meticulous preoperative assessment detected 3 large intimal tears in the descending aorta, abdominal aortic aneurysm, and left common iliac artery. We performed single-stage thoracic and abdominal endovascular aneurysm repair and concomitant axillary-axillary bypass. The abdominal aortic aneurysm with type B aortic dissection was successfully treated using a single-stage endovascular stent graft, without any complications due to the careful preoperative examinations.

  8. Aortic reconstruction with bovine pericardial grafts

    Directory of Open Access Journals (Sweden)

    Silveira Lindemberg Mota

    2003-01-01

    Full Text Available INTRODUCTION: Glutaraldehyde-treated crimped bovine pericardial grafts are currently used in aortic graft surgery. These conduits have become good options for these operations, available in different sizes and shapes and at a low cost. OBJECTIVE:To evaluate the results obtained with bovine pericardial grafts for aortic reconstruction, specially concerning late complications. METHOD: Between January 1995 and January 2002, 57 patients underwent different types of aortic reconstruction operations using bovine pericardial grafts. A total of 29 (50.8% were operated on an urgent basis (mostly acute Stanford A dissection and 28 electively. Thoracotomy was performed in three patients for descending aortic replacement (two patients and aortoplasty with a patch in one. All remaining 54 underwent sternotomy, cardiopulmonary bypass and aortic resection. Deep hypothermia and total circulatory arrest was used in acute dissections and arch operations. RESULTS: Hospital mortality was 17.5%. Follow-up was 24.09 months (18.5 to 29.8 months confidence interval and complication-free actuarial survival curve was 92.3% (standard deviation ± 10.6. Two patients lately developed thoracoabdominal aneurysms following previous DeBakey II dissection and one died from endocarditis. One "patch" aortoplasty patient developed local descending aortic pseudoaneurysm 42 months after surgery. All other patients are asymptomatic and currently clinically evaluated with echocardiography and CT scans, showing no complications. CONCLUSION: Use of bovine pericardial grafts in aortic reconstruction surgery is adequate and safe, with few complications related to the conduits.

  9. Abdominal Aortic Aneurysm (AAA)

    Science.gov (United States)

    ... News Physician Resources Professions Site Index A-Z Abdominal Aortic Aneurysm (AAA) Abdominal aortic aneurysm (AAA) occurs when atherosclerosis ... an abdominal aortic aneurysm treated? What is an abdominal aortic aneurysm? The aorta, the largest artery in the body, ...

  10. The ARCHES Project

    Science.gov (United States)

    Motch, C.; Arches Consortium

    2015-09-01

    The Astronomical Resource Cross-matching for High Energy Studies (ARCHES) project is a FP7-Space funded programme started in 2013 and involving the Observatoire Astronomique de Strasbourg including the CDS (France), the Leibniz- Institut für Astrophysik Potsdam (Germany), the University of Leicester (UK), the Universidad de Cantabria (IFCA, Spain) and the Instituto Nacional de Tecnica Aeroespacial (Spain). ARCHES will provide the international astronomical community with well-characterised multi-wavelength data in the form of spectral energy distributions (SEDs) for large samples of objects extracted from the 3XMM X-ray catalogue of serendipitous sources. The project develops new tools implementing fully probabilistic simultaneous cross-correlation of several catalogues and a multi-wavelength finder for clusters of galaxies. SEDs are based on an enhanced version of the 3XMM catalogue and on a careful selection of the most relevant multi-wavelength archival catalogues. In order to ensure the largest audience, SEDs will be distributed to the international community through CDS services and through the Virtual Observatory. These enhanced resources are tested in the framework of several science cases. More information may be found at http://www.arches-fp7.eu/

  11. Cardiovascular devices; reclassification of intra-aortic balloon and control systems for acute coronary syndrome, cardiac and non-cardiac surgery, or complications of heart failure; effective date of requirement for premarket approval for intra-aortic balloon and control systems for septic shock or pulsatile flow generation. Final order.

    Science.gov (United States)

    2013-12-30

    The Food and Drug Administration (FDA) is issuing a final order to reclassify intra-aortic balloon and control system (IABP) devices when indicated for acute coronary syndrome, cardiac and non-cardiac surgery, or complications of heart failure, a preamendments class III device, into class II (special controls), and to require the filing of a premarket approval application (PMA) or a notice of completion of a product development protocol (PDP) for IABPs when indicated for septic shock or pulsatile flow generation.

  12. Extracorporeal membrane oxygenation in children with heart disease and del22q11 syndrome: a review of the Extracorporeal Life Support Organization Registry.

    Science.gov (United States)

    Prodhan, P; Gossett, J M; Rycus, P T; Gupta, P

    2015-11-01

    The study objective was to evaluate outcomes among children with del22q11 (DiGeorge) syndrome supported on ECMO for heart disease. The ELSO registry database was queried to include all children great vessels and interrupted aortic arch and requiring ECMO, from 1998-2011. The outcomes evaluated included mortality, ECMO duration and length of hospital stay in patients with del22q11 syndrome and with no del22q11 syndrome. Eighty-eight ECMO runs occurred in children with del22q11 syndrome while 2694 ECMO runs occurred in children without del22q11 syndrome. For patients with heart defects receiving ECMO, del22q11 syndrome did not confer a significant mortality risk or an increased risk of infectious complications before or while on ECMO support. Neither the duration of ECMO nor mechanical ventilation prior to ECMO deployment were prolonged in patients with del22q11 syndrome compared to the controls.

  13. Postmortem diagnosis of Marfan syndrome in a case of sudden death due to aortic rupture: Detection of a novel FBN1 frameshift mutation.

    Science.gov (United States)

    Wang, Yunyun; Chen, Shu; Wang, Rongshuai; Huang, Sizhe; Yang, Mingzhen; Liu, Liang; Liu, Qian

    2016-04-01

    To investigate the sudden death of a 36-year-old Chinese man, a medicolegal autopsy was performed, combining forensic pathological examinations and genetic sequencing analysis to diagnose the cause of death. Genomic DNA samples were extracted from blood and subjected to high-throughput sequencing. Major findings included a dilated aortic root with a ruptured and dissected aorta and consequent tamponade of the pericardial sac. Moreover, arachnodactyly and other skeletal deformities were noted. By sequencing the fibrillin-1 gene (FBN1), five genetic variations were found, including four previously known single nucleotide polymorphisms (SNPs) and a novel frameshift mutation, leading to the diagnosis of Marfan syndrome. The frameshift mutation (c.4921delG, p.glu1641llysFsX9) detected in exon 40 led to a stop codon after the next 8 amino acids. The four SNPs included a splice site mutation (c.3464-5 G>A, rs11853943), a synonymous mutation (p.Asn625Asn, rs25458), and two missense mutations (p.Pro1148Ala, rs140598; p.Cys472Tyr, rs4775765). Genetic screening was recommended for the relatives as it was reported that the father and brother of the deceased had died at the ages of 40 and 25, respectively, from sudden cardiac failure. The son of the deceased lacked the relevant mutations. This report emphasizes the important contribution of medicolegal postmortem analysis on the molecular pathogenesis study of Marfan syndrome and early diagnosis of at-risk relatives.

  14. [Aortic valve replacement as an independent predictive factor for later development of aortic dissection].

    Science.gov (United States)

    von Kodolitsch, Y; Simic, O; Bregenzer, T; Dresler, C; Haverich, A; Nienaber, C A

    1998-08-01

    Dissection of the ascending aorta (type A) following later after aortic valve replacement has been described with increasing frequency. This study analyzes the role of aortic valve replacement for the evolution of late dissection. In a series of 80 consecutive patients with type A dissection, a previous aortic valve replacement had been performed in 12 cases (15%). In addition to arterial hypertension (p syndrome (p factor for type A dissection. Dissection occurred 3 +/- 4 years after aortic valve replacement with a clinical and anatomical profile similar to classic dissection as proven by comparison to a group of 62 patients with classic dissection associated with arterial hypertension or Marfan syndrome. With 75% and 66%, respectively, 30 day and 1 year survival of patients with dissection following later after aortic valve replacement was similar to patients with classic type A dissection. Extensive thinning and/or fragility (p associated with a high risk for late dissection; this finding was substantiated by comparison to a control group of 10 consecutive patients with a similarly dilated aortic root but no dissection. Type and diameter of valve prostheses, cross-clamp time, NYHA functional class, and left ventricular ejection fraction were unrelated to late dissection. Previous aortic valve replacement is an independent predisposing factor for a dissection of the ascending aorta later. At the time of aortic valve replacement, prophylactic replacement or wrapping of the ascending aorta should be considered in patients with a thinned/fragile aortic wall even without a markedly dilated aortic root.

  15. Combined surgical and endovascular approach to treat a complex aortic coarctation without extracorporeal circulation.

    Science.gov (United States)

    Carrel, Thierry P; Berdat, Pascal A; Baumgartner, Iris; Dinkel, Hans-Peter; Schmidli, Jürg

    2004-10-01

    Various therapeutic approaches have been proposed to treat complex coarctation of the aorta (eg, recoarctation, which requires repetitive interventions, or coarctation with a hypoplastic aortic arch). Resection followed by end-to-end anastomosis or by graft interposition is technically demanding and exposes the patient to considerable perioperative risks. Cardiopulmonary bypass and deep hypothermic circulatory arrest may be necessary to control the distal aortic arch. The role of stent technology in treating this type of lesion has not yet been defined. We present a 21-year-old woman with a recurrent coarctation of the aorta associated with a hypoplastic aortic arch and a pseudoaneurysm of the proximal descending aorta. She had undergone 4 previous interventions. Treatment consisted of a combined surgical and endovascular approach without cardiopulmonary bypass and included extraanatomic aortic bypass, partial debranching of the supraaortic vessels, and stent-graft insertion to exclude the aneurysm.

  16. HLHS with severe aortic insufficiency in a patient with 45,X/46,XY mosaicism.

    Science.gov (United States)

    Mumtaz, Muhammad A; Mee, Roger B B; Qureshi, Athar; Patel, Chandrakant R; Younoszai, Adel K

    2004-10-01

    Aortic insufficiency is not a part of the hypoplastic left heart syndrome. This report describes a rare case of congenital aortic insufficiency from a detached leaflet in a patient with hypoplastic left heart syndrome and 45,X/46XY mosaicism. The patient was subsequently treated with the modified Norwood procedure along with suture closure of aortic valve.

  17. LANGER’S AXILLARY ARCH AND ITS CLINICAL IMPLICATIONS

    Directory of Open Access Journals (Sweden)

    Jyothi K C

    2014-09-01

    Full Text Available Introduction: The axillary arch muscle of Langer is the most common anatomical variant of axillary musculature which is of clinical and surgical importance. It may lead to neurovascular compression syndrome in the cervicoaxillary region and can be misinterpreted while examining axilla and also impairs movements of shoulder joint. Observation: During routine dissection of axilla for undergraduate teaching, an unusual muscular slip in the left axilla was observed .The muscular slip was extending from lattisimus dorsi muscle to undersurface of pectoralis major muscle, arching over axillary vessels and cords of brachial plexus. Conclusion: The axillary arch may cause obstruction to axillary vessels and nerves and may be involved in thoracic outlet syndrome and shoulder instability. The knowledge of this muscular variant could help to minimize intraoperative complications related to surgeries in or nearby axilla such as mastectomy, breast reconstruction and axillary lymphadenectomy or lymph node biopsy.

  18. The ARCHES project

    CERN Document Server

    Motch, C; Genova, F; Esteban, F Jiménez-; López, M; Michel, L; Mingo, B; Mints, A; Gómez-Morán, A Nebot; Pineau, F -X; Rosen, S; Sanchez, E; Schwope, A; Solano, E; Watson, M

    2016-01-01

    ARCHES (Astronomical Resource Cross-matching for High Energy Studies) is a FP7-Space funded project whose aim is to provide the international astronomical community with well-characterised multi-wavelength data in the form of spectral energy distributions (SEDs) for large samples of objects extracted from the 3XMM DR5 X-ray catalogue of serendipitous sources. The project has developed new tools implementing fully probabilistic simultaneous cross-correlation of several catalogues for unresolved sources and a multi-wavelength finder for clusters of galaxies for extended sources. These enhanced resources have been tested in the framework of several science cases.

  19. Double arch mirror study

    Science.gov (United States)

    Vukobratovich, D.; Hillman, D.

    1983-01-01

    The development of a method of mounting light weight glass mirrors for astronomical telescopes compatible with the goals of the Shuttle Infrared Telescope Facility (SIRTF) was investigated. A 20 in. diameter double arch lightweight mirror previously fabricated was modified to use a new mount configuration. This mount concept was developed and fabricated. The mounting concept of the double mounting mirror is outlined. The modifications made to the mirror, fabrication of the mirror mount, and room temperature testing of the mirror and mount and the extension of the mirror and mount concept to a full size (40 in. diameter) primary mirror for SIRTF are discussed.

  20. Aortic coarctation, aneurysm, and ventricular dysfunction in an asymptomatic infant.

    Science.gov (United States)

    García, Ana I; Aguilar, Juan M; García, Enrique

    2016-06-01

    Aortic arch coarctation with post-coarctation aneurysm is rare in infants. We present the case of an asymptomatic 3-month-old infant with severe left ventricular dysfunction in this setting. The patient underwent surgical repair, and the left ventricular ejection fraction improved to recovery the 4th post-operative month.

  1. Endovascular treatment of late thoracic aortic aneurysms after surgical repair of congenital aortic coarctation in childhood.

    Directory of Open Access Journals (Sweden)

    Robert Juszkat

    Full Text Available BACKGROUND: In some patients, local surgery-related complications are diagnosed many years after surgery for aortic coarctation. The purposes of this study were: (1 to systematically evaluate asymptomatic adults after Dacron patch repair in childhood, (2 to estimate the formation rate of secondary thoracic aortic aneurysms (TAAs and (3 to assess outcomes after intravascular treatment for TAAs. METHODS: This study involved 37 asymptomatic patients (26 female and 11 male who underwent surgical repair of aortic coarctation in the childhood. After they had reached adolescence, patients with secondary TAAs were referred to endovascular repair. RESULTS: Follow-up studies revealed TAA in seven cases (19% (including six with the gothic type of the aortic arch and mild recoarctation in other six (16%. Six of the TAA patients were treated with stentgrafts, but one refused to undergo an endovascular procedure. In three cases, stengrafts covered the left subclavian artery (LSA, in another the graft was implanted distally to the LSA. In two individuals, elective hybrid procedures were performed with surgical bypass to the supraaortic arteries followed by stengraft implantation. All subjects survived the secondary procedures. One patient developed type Ia endoleak after stentgraft implantation that was eventually treated with a debranching procedure. CONCLUSIONS: The long-term course of clinically asymptomatic patients after coarctation patch repair is not uncommonly complicated by formation of TAAs (particularly in individuals with the gothic pattern of the aortic arch that can be treated effectively with stentgrafts. However, in some patients hybrid procedures may be necessary.

  2. A patient with ascending aortic dilatation, similar to phenotypes of connective tissue disorders.

    Science.gov (United States)

    Onrat, S T; Emmiler, M; Sivaci, Y; Söylemez, Z; Ozgöz, A; Imirzalioğlu, N

    2009-04-14

    We report on the clinical and molecular findings of a patient who presented alopecia, epicanthus, micrognathia, retrognathia, high arched palate, hypertelorism, Chiari type I malformation, mixed-type hearing loss but with normal heartbeat Q-T interval, malformed earlobes, down-slanted palpebral fissures, downturned corners of the mouth, syndactyly, atopic eczema, and seizures. The patient was a male adult, 23 years old, with short stature (153 cm) and low weight (50.5 kg), due to severe aortic insufficiency and dilatation of the ascending aorta. Conventional cytogenetic screening did not show any chromosomal gains or losses. Molecular genetic screening was conducted for gene mutations involved in various syndromes; the mutations found included [beta-fibrinogen -455 G>A wt/wt (wt/mut), PAI-1 4G/5G (4G/4G), HPA1 a/b (a/a), MTHFR C677T wt/wt (wt/mut), ACE I/D (I/I), and Apo E E3/E4]. Many clinical and molecular genetics findings overlapped with other conditions associated with arterial tortuosity and arterial aneurysms, including the Marfan, Ehler-Danlos, Shprintzen-Goldberg, and Loeys-Dietz syndromes. Although a diagnosis of Shprintzen-Goldberg syndrome was based on clinical findings and radiographic findings indicate other syndromes, aortic root dilatation seems to be a new symptom, similar to phenotypes of connective tissue disorders. The unique grouping of clinical manifestations in this patient and the molecular genetics findings lead us to suggest that this case could be an example of a previously unrecognized syndrome.

  3. Atypical Cogan's syndrome associated with coronary disease

    Institute of Scientific and Technical Information of China (English)

    Ivanovic Branislava; Tadic Marijana; Damjanov Nemanja; Simic Dragan; Zlatanovic Maja

    2011-01-01

    Cogan's syndrome (CS) is a rare inflammatory disorder characterized by interstitial keratitis and vestibuloauditory abnormalities often associated with various systemic manifestations. Involvement of cardiovascular system resembling systemic vasculitis may lead to severe complications and death. The present report describes a case of a female patient with atypical Cogan's syndrome presented with systemic manifestations and severe coronary and femoral artery stenosis.Despite the clinical improvement after glucocorticoids and cyclophosphamide, the patient required double aortocoronal bypass grafting one year letter. During three years follow-up, she was in stable condition, without stenocardial symptoms and claudication and her inflammatory parameters remain normal. This case highlights the rare involvement of coronary arteries without associated large-vessel vasculitis of the aortic arch in CS.

  4. A Clinicopathological Study on Aortic Valves in Children

    Institute of Scientific and Technical Information of China (English)

    HUANG Ping; WANG Hongwei; ZHANG Zhenlu; HU Xiufen; LI Yanping; CHENG Peixuan; LIU Jianying

    2007-01-01

    In order to investigate the clinicopathological characteristics of aortic valve disease in children, all the native surgically excised aortic valves obtained between January 2003 and December 2005 were studied macroscopically and microscopically. The patients' medical records were reviewed and the clinical information was extracted. According to preoperative echocardiography, intraoperative assessment, and postoperative pathology, combined with clinical symptoms and signs, aortic valve diseases were divided into three categories: aortic stenosis (AS), aortic insufficiency (AI), and aortic stenosis with insufficiency (AS-AI). The etiology was determined according to the macroscopic, microscopic and clinical findings. The results showed that among 70 aortic valves, patient age ranged from 6 to 18 years, with a mean of 15.4 years, and there were 56 boys and 14 girts (male: female=4:1). Forty-four children only had pure aortic valve disease, and the other 26 children had aortic valve disease associated with other heart valve diseases. There were 5 cases of AS (7.14%), 60 cases of AI (85.71%) and 5 cases of AS-AI (7.14%). The causes were congenital aortic valve malformation (32 cases, 45.71%), rheumatic disease (28 cases, 40%), infective endocarditis (7 cases,10%), Marfan syndrome (2 cases, 2.86%), and undetermined (1 case, 1.43%). It was concluded that the common causes of aortic valve disease in order of frequency in children were congenital aortic valve malformation, rheumatic disease, infective endocarditis, and Marfan syndrome. AI was more common in children with aortic valve disease. Compared with adult patients, congenital bicuspid aortic valve in children was often AI. Histologically, the leaflets of congenital bicuspid aortic valve were mainly myxomatous, fibrosis and calcification less seen. AI was frequently found in rheumatic disease, mostly associated with other heart valve diseases. Macroscopic and microscopic examinations together with clinical

  5. 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.

  6. Aortic Blood Flow Reversal Determines Renal Function: Potential Explanation for Renal Dysfunction Caused by Aortic Stiffening in Hypertension.

    Science.gov (United States)

    Hashimoto, Junichiro; Ito, Sadayoshi

    2015-07-01

    Aortic stiffness determines the glomerular filtration rate (GFR) and predicts the progressive decline of the GFR. However, the underlying pathophysiological mechanism remains obscure. Recent evidence has shown a close link between aortic stiffness and the bidirectional (systolic forward and early diastolic reverse) flow characteristics. We hypothesized that the aortic stiffening-induced renal dysfunction is attributable to altered central flow dynamics. In 222 patients with hypertension, Doppler velocity waveforms were recorded at the proximal descending aorta to calculate the reverse/forward flow ratio. Tonometric waveforms were recorded to measure the carotid-femoral (aortic) and carotid-radial (peripheral) pulse wave velocities, to estimate the aortic pressure from the radial waveforms, and to compute the aortic characteristic impedance. In addition, renal hemodynamics was evaluated by duplex ultrasound. The estimated GFR was inversely correlated with the aortic pulse wave velocity, reverse/forward flow ratio, pulse pressure, and characteristic impedance, whereas it was not correlated with the peripheral pulse wave velocity or mean arterial pressure. The association between aortic pulse wave velocity and estimated GFR was independent of age, diabetes mellitus, hypercholesterolemia, and antihypertensive medication. However, further adjustment for the aortic reverse/forward flow ratio and pulse pressure substantially weakened this association, and instead, the reverse/forward flow ratio emerged as the strongest determinant of estimated GFR (P=0.001). A higher aortic reverse/forward flow ratio was also associated with lower intrarenal forward flow velocities. These results suggest that an increase in aortic flow reversal (ie, retrograde flow from the descending thoracic aorta toward the aortic arch), caused by aortic stiffening and impedance mismatch, reduces antegrade flow into the kidney and thereby deteriorates renal function.

  7. Residual glycosaminoglycan accumulation in mitral and aortic valves of a patient with attenuated MPS I (Scheie syndrome after 6 years of enzyme replacement therapy: Implications for early diagnosis and therapy

    Directory of Open Access Journals (Sweden)

    Yohei Sato

    2015-12-01

    Full Text Available Mucopolysaccharidosis (MPS is an inherited metabolic disease caused by deficiency of the enzymes needed for glycosaminoglycan (GAG degradation. MPS type I is caused by the deficiency of the lysosomal enzyme alpha-l-iduronidase and is classified into Hurler syndrome, Scheie syndrome, and Hurler–Scheie syndrome based on disease severity and onset. Cardiac complications such as left ventricular hypertrophy, cardiac valve disease, and coronary artery disease are often observed in MPS type I. Enzyme replacement therapy (ERT has been available for MPS type I, but the efficacy of this treatment for cardiac valve disease is unknown. We report on a 56-year-old female patient with attenuated MPS I (Scheie syndrome who developed aortic and mitral stenosis and coronary artery narrowing. The cardiac valve disease progressed despite ERT and she finally underwent double valve replacement and coronary artery bypass grafting. The pathology of the cardiac valves revealed GAG accumulation and lysosomal enlargement in both the mitral and aortic valves. Zebra body formation was also confirmed using electron microscopy. Our results suggest that ERT had limited efficacy in previously established cardiac valve disease. Early diagnosis and initiation of ERT is crucial to avoid further cardiac complications in MPS type I.

  8. Early detection of Berry syndrome in a newborn with differential cyanosis

    Institute of Scientific and Technical Information of China (English)

    FONG Nai-chung; KONG Chun-tat; MAK Wai-yau; SHIU Yiu-keung; LEE Shing-yan; CHOW Chun-bong; CHIU Man-chun

    2006-01-01

    @@ Berry Syndrome is a rare combination of congenital cardiac abnormalities firstly reported in 1982.1 It consists of aortopulmonary window, anomalous origin of the right pulmonary artery (RPA) from ascending aorta, intact ventricular septum,interruption of the aortic arch with patent ductus arteriosus(PDA). This is the 26th case reported in literature2 and the first report in Hong Kong. Delayed recognition can result in potential lethal condition.This report demonstrated the importance of prompt clinical recognition, timely echocardiography and early operation in the management of this rare cardiac anomaly.

  9. The First Case Report in Italy of Di George Syndrome Detected by Noninvasive Prenatal Testing

    Directory of Open Access Journals (Sweden)

    Giuseppina Rapacchia

    2015-01-01

    Full Text Available Panorama Plus (Natera, a single-nucleotide polymorphism- (SNP- based approach that relies on the identification of maternal and fetal allele distributions, allows the detection of common aneuploidies and also incorporates a panel of 5 microdeletions including Di George syndrome. We report here the first case of Di George syndrome detected by NIPT in Italy; blood was drawn at 12 weeks’ gestation. The patient had an amniocentesis to confirm the diagnosis by MLPA (multiplex ligation-dependent probe amplification and an ultrasound aimed to detect the features associated with the syndrome. A right aortic arch and suspect of thymus atrophy were detected, but not other severe malformations typical of the disease. The patient terminated the pregnancy at 17 weeks. NIPT allowed an early screening of Di George syndrome. As the patient was at low risk, it is likely that an ultrasound would have missed the condition.

  10. The First Case Report in Italy of Di George Syndrome Detected by Noninvasive Prenatal Testing

    Science.gov (United States)

    Rapacchia, Giuseppina; Lapucci, Cristina; Pittalis, Maria Carla; Youssef, Aly; Farina, Antonio

    2015-01-01

    Panorama Plus (Natera), a single-nucleotide polymorphism- (SNP-) based approach that relies on the identification of maternal and fetal allele distributions, allows the detection of common aneuploidies and also incorporates a panel of 5 microdeletions including Di George syndrome. We report here the first case of Di George syndrome detected by NIPT in Italy; blood was drawn at 12 weeks' gestation. The patient had an amniocentesis to confirm the diagnosis by MLPA (multiplex ligation-dependent probe amplification) and an ultrasound aimed to detect the features associated with the syndrome. A right aortic arch and suspect of thymus atrophy were detected, but not other severe malformations typical of the disease. The patient terminated the pregnancy at 17 weeks. NIPT allowed an early screening of Di George syndrome. As the patient was at low risk, it is likely that an ultrasound would have missed the condition. PMID:26346617

  11. Using ArchE in the Classroom: One Experience

    Science.gov (United States)

    2007-09-01

    displaying the new itinerary The team initially adopted the Model-View-Controller ( MVC ) architecture as the top-level architecture . The...CMU/SEI-2007-TN-001 Software Architecture Technology Initiative Unlimited distribution subject to the copyright. This report was prepared...SOFTWARE ENGINEERING INSTITUTE | v vi | CMU/SEI-2007-TN-001 Abstract The Architecture Expert (ArchE) tool serves as a software architecture

  12. Aortic Stenosis.

    Science.gov (United States)

    Bakaeen, Faisal G; Rosengart, Todd K; Carabello, Blase A

    2017-01-03

    This issue provides a clinical overview of aortic stenosis, focusing on screening, diagnosis, treatment, and practice improvement. The content of In the Clinic is drawn from the clinical information and education resources of the American College of Physicians (ACP), including MKSAP (Medical Knowledge and Self-Assessment Program). Annals of Internal Medicine editors develop In the Clinic in collaboration with the ACP's Medical Education and Publishing divisions and with the assistance of additional science writers and physician writers.

  13. Effects of Aortic Irregularities on the Blood Flow

    Science.gov (United States)

    Gutmark-Little, Iris; Prahl-Wittberg, Lisa; van Wyk, Stevin; Mihaescu, Mihai; Fuchs, Laszlo; Backeljauw, Philippe; Gutmark, Ephraim

    2013-11-01

    Cardiovascular defects characterized by geometrical anomalies of the aorta and its effect on the blood flow are investigated. The flow characteristics change with the aorta geometry and the rheological properties of the blood. Flow characteristics such as wall shear stress often play an important role in the development of vascular disease. In the present study, blood is considered to be non-Newtonian and is modeled using the Quemada model, an empirical model that is valid for different red blood cell loading. Three patient-specific aortic geometries are studied using Large Eddy Simulations (LES). The three geometries represent malformations that are typical in patients populations having a genetic disorder called Turner syndrome. The results show a highly complex flow with regions of recirculation that are enhanced in two of the three aortas. Moreover, blood flow is diverted, due to the malformations, from the descending aorta to the three side branches of the arch. The geometry having an elongated transverse aorta has larger areas of strong oscillatory wall shear stress.

  14. The protective effects of edaravone to cerebral ischemia reperfusion injury during aortic arch replacement surgery%依达拉奉对主动脉弓部手术深低温停循环脑缺血再灌注损伤的保护作用

    Institute of Scientific and Technical Information of China (English)

    赵文度; 卿恩明; 卢家凯; 程卫平; 邱莉

    2012-01-01

    Objective:To investigate the protective effects of edaravone to cerebral ischemia reperfusion injury for patients undergoing deep hypothermic circulatory arrest (DHCA) during great artery surgery. Methods; A total of 60 patients undergoing total aortic arch replacement combined with elephant trunk implantation from to were recruited. They were randomly divided into two groups; edaravone group (Group Y, n = 30) received edaravone 0. 5 mg/kg in normal solution, and control group (Croup C, n =30) received placebo. Two groups were inducted and maintained by the same anesthetic medications. During the operation, the anesthesia medications were dosed in the same way. Blood samples were collected from intrajugular vein for determination of serum levels of super oxide dismutase ( SOD), malondialdehyde ( MDA) , neuron-specific cenolase( NSE) and total antioxidant capacity (TAOC) at following 6 time-points, before the beginning of surgery (T1) , after cessation of DHCA for 5min after (T2), 1h (T3), 2h (T4) ,6h (T5) and 24h (T6). Results: There were no significant differences in serum levels of SOD ,MDA ,NSE and TAOC between the two groups at point of T1 The serum levels of NSE., MDA and TAOC were significantly increased at points after cessation of DHCA ( T2-5 ) compared to the value at point of T1. SOD activity and TAOC at points of cessation of DHCA (T2-5) were significantly higher in the experimental group than those in the control group(P <0.05 or 0.01). The serum levels of NSE and MDA after cessation of DHCA (T2-5) were significantly lower in the experiment group than those in the control group( P < 0.05, P < 0. 01). Conclusion; Edaravone can attenuate cerebral injury during aorticsurgery with DHCA. Edaravone used in aortic surgery with DHCA can eliminate OFR effectively, protect the activity of SOD, increase the activity of TAOC and decrease production of MDA, which can reduce the cerebral ischemia-reperfusion injury and attenuate cerebral injury. Therefore, edaravone

  15. Marfan syndrome

    Science.gov (United States)

    ... at least once every year. Alternative Names Aortic aneurysm - ... syndrome. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 702. ...

  16. Aortic valve bypass

    DEFF Research Database (Denmark)

    Lund, Jens T; Jensen, Maiken Brit; Arendrup, Henrik;

    2013-01-01

    In aortic valve bypass (AVB) a valve-containing conduit is connecting the apex of the left ventricle to the descending aorta. Candidates are patients with symptomatic aortic valve stenosis rejected for conventional aortic valve replacement (AVR) or transcatheter aortic valve implantation (TAVI...

  17. Abdominal aortic calcification quantified by the Morphological Atherosclerotic Calcification Distribution (MACD) index is associated with features of the metabolic syndrome

    DEFF Research Database (Denmark)

    Barascuk, Natasha; Ganz, Melanie; Nielsen, Mads;

    2011-01-01

    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....... At baseline and across all patients, MACD correlated with blood glucose (r2 = 0.1, Prisk factors (p .... 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...

  18. 产前超声诊断胎儿主动脉闭锁%Prenatal ultrasound diagnosis of fetal aortic atresia

    Institute of Scientific and Technical Information of China (English)

    许燕; 何敬海; 接连利; 程建; 高翔; 刘清华

    2012-01-01

    目的 探讨产前超声诊断胎儿主动脉闭锁的临床价值.方法 回顾性分析9例经引产后尸检证实的主动脉闭锁胎儿的产前超声心动图表现,总结其声像图特征.结果 9例主动脉闭锁胎儿声像图显示升主动脉及主动脉弓细窄7例,升主动脉及主动脉弓显示不清2例,彩色多普勒血流显像9例胎儿均显示主动脉与左心室无血流连接,主动脉弓显示反向血流.9例胎儿中6例伴发左心发育不良,2例伴发室间隔缺损,左心室发育良好,1例伴发完全型房室间隔缺损.9例胎儿检出时平均孕龄(26.0±2.6)周,孕龄最小者仅为孕16周.结论 产前超声诊断胎儿主动脉闭锁具有重要临床价值;升主动脉、主动脉弓细窄或显示不清,主动脉与左心室无血流连接及主动脉弓内反向血流是主要诊断依据;彩色多普勒血流显像有助于早期诊断.%Objective To investigate the clinical value of prenatal ultrasonography in diagnosing fetal aortic atresia.Methods Ultrasound images of 9 cases with fetal aortic atresia confirmed by autopsy were retrospectively analysed and summarized.Results Fetal echocardiography showed ascending aorta and aortic arch were thin in seven cases and unclear in two cases.Color Doppler flow imaging showed fetal aorta no blood connection with left ventricle and reverse blood flow in aortic arch among all 9 cases.There were 6 cases associated with hypoplastic left heart syndrome,two cases associated with ventricular septal defect and well-developed left ventricle,one case complicated with complete atrioventricular septal defect.Nine cases were detected at average gestational age (26.0 ± 2.6) weeks,minimum gestational age was only 16 weeks pregnant.Conclusions Prenatal ultrasound diagnosis of fetal aortic atresia has important clinical value.Ascending aorta and aortic arch are thin or unclear,no blood flow connection between fetal aorta and left ventricle and reverse blood flow in aortic arch are

  19. Type B Aortic Dissection: Management Updates

    Directory of Open Access Journals (Sweden)

    Naghmeh Moshtaghi

    2009-12-01

    Full Text Available Acute aortic dissection (AAD is the most frequent catastrophic event of the aorta; it occurs nearly three times as frequently as the rupture of abdominal aortic aneurysm. Sixty percent of dissection cases are classified as proximal or type A and 40% as distal or type B, according to the Stanford Classification. The most frequent causes of death in acute type B dissection are aortic rupture and malperfusion syndrome.We herein review recent data suggesting different management modalities of type B aortic dissection, including medical, surgical, and endovascular treatments. Although medical therapy is still the standard approach in uncomplicated cases, there are subgroups of patients who may benefit from endovascular management. Endovascular techniques or surgery are valuable options for complicated cases. Hybrid suites, multidisciplinary approaches, and good imaging techniques can be considered as the key to success in this regard.

  20. New technique for single-staged repair of aortic coarctation and coexisting cardiac disorder.

    Science.gov (United States)

    Korkmaz, Askin Ali; Guden, Mustafa; Onan, Burak; Tarakci, Sevim Indelen; Demir, Ali Soner; Sagbas, Ertan; Sarikaya, Tugay

    2011-01-01

    The management of adults with aortic coarctation and a coexisting cardiac disorder is still a surgical challenge. Single-staged procedures have lower postoperative morbidity and mortality rates than do 2-staged procedures. We present our experience with arch-to-descending aorta bypass grafting in combination with intracardiac or ascending aortic aneurysm repair.From October 2004 through April 2010, 5 patients (4 men, 1 woman; mean age, 45.8 ± 9.4 yr) underwent anatomic bypass grafting of the arch to the descending aorta through a median sternotomy and concomitant repair of an intracardiac disorder or an ascending aortic aneurysm. Operative indications included coarctation of the aorta in all cases, together with severe mitral insufficiency arising from damaged chordae tendineae in 2 patients, ascending aortic aneurysm with aortic regurgitation in 2 patients, and coronary artery disease in 1 patient. Data from early and midterm follow-up were reviewed.There was no early or late death. Follow-up was complete for all patients, and the mean follow-up period was 34.8 ± 18 months (range, 18 mo-5 yr). All grafts were patent. No late graft-related sequelae or reoperations were observed.For single-staged repair of aortic coarctation with a coexistent cardiac disorder, we propose arch-to-descending aorta bypass through a median sternotomy as an alternative for selected patients.

  1. Theory of Arched Structures Strength, Stability, Vibration

    CERN Document Server

    Karnovsky, Igor A

    2012-01-01

    Theory of Arched Structures: Strength, Stability, Vibration presents detailed procedures for analytical analysis of the strength, stability, and vibration of arched structures of different types, using exact analytical methods of classical structural analysis. The material discussed is divided into four parts. Part I covers stress and strain with a particular emphasis on analysis; Part II discusses stability and gives an in-depth analysis of elastic stability of arches and the role that matrix methods play in the stability of the arches; Part III presents a comprehensive tutorial on dynamics and free vibration of arches, and forced vibration of arches; and Part IV offers a section on special topics which contains a unique discussion of plastic analysis of arches and the optimal design of arches.

  2. MARFAN SYNDROME – A CONTINUOUS MULTIDISCIPLINARY CHALLENGE

    Directory of Open Access Journals (Sweden)

    Paloma MANEA

    2012-06-01

    Full Text Available The paper discusses the clinical case of a 26 year-old pacient, diagnosed, as early as the age of 10 years, with a valvular pathology (at that time, objective reasons preventing the development ofadditional investigations on its etiology and recommended treatment. In January 2012, the patient suffers two tachyarhythmic episodes, during a longer trip, while driving his car and, next February, a routine stomatological exam evidences an ogival palatine arch and malar hypoplasia, so that a cardiological consultation follows. Clinical examination revealed a longiline status, arachnodactylia, pectus excavatum and telesystolic mitral murmur. Echocardiographic examination evidences prolapse of anterior mitral valve and mild secondary anterior mitral regurgitation, dilatation of the ascending aorta and mild aortic regurgitation. The suspicion of Marfan syndrome, based on the Ghent criteria, revised in 2010, was confirmed. Treatment with betablocking drugs (Bisoprolol was recommended. Patient’s evolution was favourable.

  3. Arch bridges – unlocking their potential

    OpenAIRE

    Long, Adrian; Nanukuttan, Sreejith

    2016-01-01

    Arch bridges are strong, durable, aesthetically pleasing and require little maintenance but very few have been built since the early 1900s. However, this trend has changed as more than 60 FlexiArch bridges have been installed since the system was launched in 2007. The FlexiArch uses precast concrete voussoirs, requires neither corrodible reinforcement, nor centring, can be installed in hours and is contractor friendly. Details of this innovative method of construction and installation of arch...

  4. The use of a new hybrid stentgraft for the repair of extensive thoracic aortic aneurysms with the frozen elephant trunk method – first Polish experiences

    OpenAIRE

    Zembala, Michał; Krasoń, Marcin; Hrapkowicz, Tomasz; Przybylski, Roman; Filipiak, Krzysztof; Borowicz, Marcin; Niklewski, Tomasz; Głowacki, Jan; Wolny, Tomasz; Nadziakiewicz, Paweł; Walas, Ryszard; Zembala, Marian

    2014-01-01

    The frozen elephant trunk (FET) technique is a modification of the traditional elephant trunk method, which was introduced by Borst in 1983 in order to treat extensive thoracic aortic aneurysms. The crux of the new method is the different type of aortic prosthesis, consisting of a Dacron part (with or without branches leading to the arterial vessels which exit the aortic arch) and a port for extracorporeal circulation with a self-expanding nitinol stentgraft. This combination enables a comple...

  5. Aortic Annular Enlargement during Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Selman Dumani

    2016-09-01

    Full Text Available In the surgery of aortic valve replacement is always attempted, as much as possible, to implant the larger prosthesis with the mains goals to enhance the potential benefits, to minimise transvalvular gradient, decrease left ventricular size and avoid the phenomenon of patient-prosthesis mismatch. Implantation of an ideal prosthesis often it is not possible, due to a small aortic annulus. A variety of aortic annulus enlargement techniques is reported to avoid patient-prosthesis mismatch. We present the case that has submitted four three times open heart surgery. We used Manouguian technique to enlarge aortic anulus with excellent results during the fourth time of surgery.

  6. Aortic aneurysm repair - endovascular

    Science.gov (United States)

    EVAR; Endovascular aneurysm repair - aorta; AAA repair - endovascular; Repair - aortic aneurysm - endovascular ... Endovascular aortic repair is done because your aneurysm is very large, growing quickly, or is leaking or bleeding. You may have ...

  7. Abdominal aortic aneurysm

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000162.htm Abdominal aortic aneurysm To use the sharing features on this page, ... blood to the abdomen, pelvis, and legs. An abdominal aortic aneurysm occurs when an area of the aorta becomes ...

  8. Severe aortic valve stenosis and nosebleed

    NARCIS (Netherlands)

    Schoedel, Johannes; Obergfell, Achim; Maass, Alexander H.; Schodel, Johannes

    2007-01-01

    Aortic valve stenosis is known to be associated with loss of high molecular von Willebrand multimers. This can lead to gastrointestinal bleeding in patients with gastrointestinal angiodysplasia, the Heyde syndrome. Here we present a case of anaemia and severe epistaxis associated with acquired von W

  9. Aortic growth rates in chronic aortic dissection

    Energy Technology Data Exchange (ETDEWEB)

    Kelly, A.M. [Department of Radiology, Division of Thoracic Radiology, University of Michigan Medical Center (United States)]. E-mail: ainekell@med.umich.edu; Quint, L.E. [Department of Radiology, Division of Thoracic Radiology, University of Michigan Medical Center (United States); Nan, B. [School of Public Health, University of Michigan, Ann Arbor, Michigan (United States); Zheng, J. [School of Public Health, University of Michigan, Ann Arbor, Michigan (United States); Cronin, P. [Department of Radiology, Division of Thoracic Radiology, University of Michigan Medical Center (United States); Deeb, G.M. [Division of Cardiac Surgery, University of Michigan Medical Center (United States); Williams, D.M. [Division of Vascular Interventional Imaging, University of Michigan Medical Center (United States)

    2007-09-15

    Aim: To determine and compare rates of descending aortic enlargement and complications in chronic aortic dissection with and without a proximal aortic graft. Methods and materials: Fifty-two patients with dissection involving the descending aorta and who had undergone at least two computed tomography (CT) examinations at our institution between November, 1993 and February, 2004 were identified, including 24 non-operated patients (four type A, 20 type B) and 28 operated patients (type A). CT examinations per patient ranged from two to 10, and follow-up ranged from 1-123 months (mean 49 months, median 38.5 months). On each CT image, the aortic short axis (SA), false lumen (FL), and true lumen (TL) diameters were measured at the longitudinal midpoint of the dissection and at the point of maximum aortic diameter. Complications were tabulated, including aortic rupture and aortic enlargement requiring surgery. Results: For non-operated patients, the midpoint and maximum point SA, TL, and FL diameters increased significantly over time. For operated patients, the midpoint and maximum point SA and FL diameters increased significantly over time. In both groups, aortic enlargement was predominantly due to FL expansion. Diameter increases in non-operated patients were significantly larger than those in operated patients. The rate of change in aortic diameter was constant, regardless of aortic size. Four non-operated and six operated patients developed aortic complications. Conclusions: In patients with a dissection involving the descending thoracic aorta, the FL increased in diameter over time, at a constant rate, and to a greater degree in non-operated patients (mostly type B) compared with operated patients (all type A)

  10. Aortic Coarctation Diagnosed During Pregnancy in a Woman With Repaired Tetralogy of Fallot.

    Science.gov (United States)

    Jalal, Zakaria; Iriart, Xavier; Thambo, Jean-Benoit

    2015-09-01

    Aortic coarctation is thought to be a rare condition in patients with tetralogy of Fallot. We report the case of a 26 year old woman presenting with systemic hypertension at 17 weeks of pregnancy after repair of tetralogy of Fallot in childhood. Echocardiography and magnetic resonance imaging revealed right aortic arch with severe isthmic coarctation. Her blood pressure was controlled medically during the rest of her pregnancy, and delivery was uneventful. Successful transcatheter placement of a covered stent at the level of the coarctation was performed after delivery. To our knowledge, this is the first reported case of aortic coarctation diagnosed in an adult patient late after repair of tetralogy of Fallot.

  11. Repair of Late Retrograde Type A Aortic Dissection After TEVAR: Causes and Management.

    Science.gov (United States)

    Mosquera, Victor X; Marini, Milagros; Fraga-Manteiga, Daniel; Gulias, Daniel; Cuenca, Jose J

    2016-03-01

    One of the most feared complications of thoracic endovascular aortic repair (TEVAR) and hybrid arch repair is retrograde type A aortic dissection (RTAD). More than two-thirds of RTAD occurs in the immediate postoperative period and first postoperative month. In presentations beyond that point, progression of the native aortopathy must be considered. We report a late presentation of an RTAD seven months after hybrid repair of an aortic intramural hematoma with an ulcer-like projection, and review the causes and management of this TEVAR complication.

  12. Endothelium in the pharyngeal arches 3, 4 and 6 is derived from the second heart field.

    Science.gov (United States)

    Wang, Xia; Chen, Dongying; Chen, Kelley; Jubran, Ali; Ramirez, AnnJosette; Astrof, Sophie

    2017-01-15

    Oxygenated blood from the heart is directed into the systemic circulation through the aortic arch arteries (AAAs). The AAAs arise by remodeling of three symmetrical pairs of pharyngeal arch arteries (PAAs), which connect the heart with the paired dorsal aortae at mid-gestation. Aberrant PAA formation results in defects frequently observed in patients with lethal congenital heart disease. How the PAAs form in mammals is not understood. The work presented in this manuscript shows that the second heart field (SHF) is the major source of progenitors giving rise to the endothelium of the pharyngeal arches 3 - 6, while the endothelium in the pharyngeal arches 1 and 2 is derived from a different source. During the formation of the PAAs 3 - 6, endothelial progenitors in the SHF extend cellular processes toward the pharyngeal endoderm, migrate from the SHF and assemble into a uniform vascular plexus. This plexus then undergoes remodeling, whereby plexus endothelial cells coalesce into a large PAA in each pharyngeal arch. Taken together, our studies establish a platform for investigating cellular and molecular mechanisms regulating PAA formation and alterations that lead to disease.

  13. Giant Aneursym of the Ascending Aorta 37 Years after Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Fabrizio Sansone

    2013-04-01

    Full Text Available Giant ascending aorta aneurysms (AAA, which are larger than 10 cm, are rare. We hereby present the case of a giant AAA of about 13 cm, incidentally detected several years after aortic valve replacement and treated according to the Cabrol technique without postoperative complications. [Arch Clin Exp Surg 2013; 2(2.000: 129-131

  14. 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 ...

  15. Comparison between different plantar arch

    OpenAIRE

    2009-01-01

    The longitudinal plantar arch (LPA) perform biomechanics functions in the foot. The aim of this study was to compare the relationship between different index to measure the LPA of the foot. The arc index, Staheli index, Viladot classification and Feiss line were compared. In the transversal study, the experimental samples were soccer players females with age between 13 to 19 years. The measure were performed by plantigraphy, with out Feiss line. Although non significant the higher agreement w...

  16. The Arches Cluster Mass Function

    CERN Document Server

    Kim, S S; Kudritzki, R P; Najarro, F; Kim, Sungsoo S.; Figer, Donald F.; Kudritzki, Rolf P.

    2006-01-01

    We have analyzed H and K_s-band images of the Arches cluster obtained using the NIRC2 instrument on Keck with the laser guide star adaptive optics (LGS AO) system. With the help of the LGS AO system, we were able to obtain the deepest ever photometry for this cluster and its neighborhood, and derive the background-subtracted present-day mass function (PDMF) down to 1.3 Msun for the 5 arcsec-9 arcsec annulus of the cluster. We find that the previously reported turnover at 6 Msun is simply due to a local bump in the mass function (MF), and that the MF continues to increase down to our 50 % completeness limit (1.3 Msun) with a power-law exponent of Gamma = -0.91 for the mass range of 1.3 < M/Msun < 50. Our numerical calculations for the evolution of the Arches cluster show that the Gamma values for our annulus increase by 0.1-0.2 during the lifetime of the cluster, and thus suggest that the Arches cluster initially had Gamma of -1.0 ~ -1.1, which is only slightly shallower than the Salpeter value.

  17. Surgical Management of Ascending Aortic Aneurysm and Its Complications

    Directory of Open Access Journals (Sweden)

    Sisira Sran

    2014-01-01

    Full Text Available Ascending aortic aneurysms involving the proximal aortic arch, arising anywhere from the aortic valve to the innominate artery, represent various problems in which open surgery is generally required. Surgical options include excision of the aortic pathology or wrapping the aneurysm shell with an aortic Dacron graft. Intervention using the latter method can lead to extravasation of blood along the suture lines resulting in continuous bleeding within the periprosthetic space. The Cabrol technique was developed as a method for decompression of postoperative leaks by the formation of a conduit system from the periprosthetic space to the right atrium. The coronary ostia are anastomosed to a second graft in an end-to-end fashion, which is then anastomosed to the ascending aortic conduit side to side. The native aorta is then sewn around the prosthesis, hereby creating a shunt to drain anastomotic leakage. This shunt reduces postsurgical risk of pseudoaneurysm formation and normally closes a few days following surgery. We discuss the case of a patient who underwent Cabrol’s variation and six months later was demonstrated to have a patent shunt.

  18. Effect of age on aortic atherosclerosis

    Institute of Scientific and Technical Information of China (English)

    Michael A. Chen; Miwa Kawakubo; Patrick M. Colletti; Dongxiang Xu; Laurie LaBree Dustin; Robert Detrano; Stanley P Azen; Nathan D. Wong; Xue-Qiao Zhao

    2013-01-01

    Objective To examine the association of atherosclerosis burden in the survivors of an asymptomatic elderly cohort study and its relationship to other coronary risk factors (specifically, age) by evaluating aortic atherosclerotic wall burden by magnetic resonance imaging (MRI). Methods A total of 312 participants in an ongoing observational cohort study underwent cardiac and descending thoracic aorta imaging by MRI. Maximum wall thickness was measured and the mean wall thickness calculated. Wall/outer wall ratio was used as a normalized wall index (NWI) adjusted for artery size difference among participants. Percent wall volume (PWV) was calculated as NWI × 100. Results In this asymptomatic cohort (mean age: 76 years), the mean (SD) aortic wall area and wall thickness were 222 ± 45 mm2 and 2.7 ± 0.4 mm, respectively. Maximum wall thickness was 3.4 ± 0.6 mm, and PWV was 32% ± 4%. Women appeared to have smaller wall area, but after correcting for their smaller artery size, had significantly higher PWV than men (P = 0.03). Older age was associated with larger wall area (P = 0.04 for trend) with similar PWVs. However, there were no statistically significant associations between standard risk factors, Framingham global risk, or metabolic syndrome status, therapy for cholesterol or hypertension, coronary or aortic calcium score, and the aortic wall burden. Aortic calcification was associated with coronary calcification. Conclusions Asymptomatic elderly in this cohort had a greater descending thoracic aortic wall volume that correlated with age, and women had a significantly increased PWV compared to men. In these survivors, the atherosclerotic aortic wall burden was not significantly associated with traditional risk factors or with coronary or aortic calcium scores or coronary calcium progression. Results suggest that age, or as yet unidentified risk factor(s), may be responsible for the increase in atherosclerosis.

  19. FBN1 Mutations in Patients With Descending Thoracic Aortic Dissections

    Science.gov (United States)

    Brautbar, Ariel; LeMaire, Scott A.; Franco, Luis M.; Coselli, Joseph S.; Milewicz, Dianna M.; Belmont, John W.

    2013-01-01

    Aortic aneurysm and dissection cause significant morbidity and mortality. There are several known single gene disorders that predispose to isolated aortic disease and eventually aneurysm and dissection. FBN1 mutations are associated with multiple clinical phenotypes, including Marfan syndrome (MFS), MASS phenotype, and familial ectopia lentis, but rarely with isolated aortic aneurysm and dissection. In this report, we describe three patients who presented with primary descending thoracic aortic dissection and who were found to have an FBN1 mutation. None of the patients fulfilled clinical criteria for the diagnosis of MFS, and all had few or none of the skeletal features typical of the condition. Two patients had a history of long-term hypertension, and such a history was suspected in the third patient. These observations suggest that some individuals with FBN1 mutations have significant aortic disease involvement of other systems that is typical of FBN1 mutation-related syndromes. Superimposed risk factors, such as hypertension, may weaken the aortic wall and eventually lead to aortic dissection. Given that the cost continues to decrease, we suggest that diagnostic DNA sequencing for FBN1 mutations in patients with thoracic aortic aneurysms and dissection may be a practical clinical step in evaluating such patients and at-risk family members. PMID:20082464

  20. The use of a new hybrid stentgraft for the repair of extensive thoracic aortic aneurysms with the frozen elephant trunk method - first Polish experiences.

    Science.gov (United States)

    Zembala, Michał; Krasoń, Marcin; Hrapkowicz, Tomasz; Przybylski, Roman; Filipiak, Krzysztof; Borowicz, Marcin; Niklewski, Tomasz; Głowacki, Jan; Wolny, Tomasz; Nadziakiewicz, Paweł; Walas, Ryszard; Zembala, Marian

    2014-09-01

    The frozen elephant trunk (FET) technique is a modification of the traditional elephant trunk method, which was introduced by Borst in 1983 in order to treat extensive thoracic aortic aneurysms. The crux of the new method is the different type of aortic prosthesis, consisting of a Dacron part (with or without branches leading to the arterial vessels which exit the aortic arch) and a port for extracorporeal circulation with a self-expanding nitinol stentgraft. This combination enables a complete one-stage treatment of the pathologies within the arch and the proximal segment of the descending aorta; moreover, it facilitates the performance of a two-stage hybrid treatment of extensive thoracic aortic aneurysms involving a significant part of the descending aorta. This article presents the cases of four patients with extensive aortic disease, who were implanted with Thoraflex prostheses (Vascutek, Scotland).

  1. The use of a new hybrid stentgraft for the repair of extensive thoracic aortic aneurysms with the frozen elephant trunk method – first Polish experiences

    Science.gov (United States)

    Krasoń, Marcin; Hrapkowicz, Tomasz; Przybylski, Roman; Filipiak, Krzysztof; Borowicz, Marcin; Niklewski, Tomasz; Głowacki, Jan; Wolny, Tomasz; Nadziakiewicz, Paweł; Walas, Ryszard; Zembala, Marian

    2014-01-01

    The frozen elephant trunk (FET) technique is a modification of the traditional elephant trunk method, which was introduced by Borst in 1983 in order to treat extensive thoracic aortic aneurysms. The crux of the new method is the different type of aortic prosthesis, consisting of a Dacron part (with or without branches leading to the arterial vessels which exit the aortic arch) and a port for extracorporeal circulation with a self-expanding nitinol stentgraft. This combination enables a complete one-stage treatment of the pathologies within the arch and the proximal segment of the descending aorta; moreover, it facilitates the performance of a two-stage hybrid treatment of extensive thoracic aortic aneurysms involving a significant part of the descending aorta. This article presents the cases of four patients with extensive aortic disease, who were implanted with Thoraflex prostheses (Vascutek, Scotland). PMID:26335241

  2. Aortic dissection: magnetic resonance imaging.

    Science.gov (United States)

    Amparo, E G; Higgins, C B; Hricak, H; Sollitto, R

    1985-05-01

    Fifteen patients with suspected or known aortic dissection were imaged with magnetic resonance (MR). Thirteen of these patients were eventually shown to have dissection. In most instances the diagnosis was established by aortography and/or computed tomography (CT) prior to the MR study. Surgical proof (6/13) and/or aortographic proof (10/13) were available in 11/13 patients with aortic dissection. MR demonstrated the intimal flap and determined whether the dissection was type A or type B. In addition, MR: differentiated between the true and false lumens; determined the origins of the celiac, superior mesenteric, and renal arteries from the true or false lumen in the cases where the dissection extended into the abdominal aorta (8/12); allowed post-surgical surveillance of the dissection; and identified aortoannular ectasia in the three patients who had Marfan syndrome. In addition to the 13 cases with dissection, there were two cases in whom the diagnosis of dissection was excluded by MR. Our early experience suggests that MR can serve as the initial imaging test in clinically suspected cases of aortic dissection and that the information provided by MR is sufficient to manage many cases. Additionally, MR obviates the use of iodinated contrast media.

  3. Palliative stent graft placement combined with subsequent open surgery for retrograde ascending dissection intra-thoracic endovascular aortic repair

    OpenAIRE

    Zhu, Kai; Lai, Hao; Guo, Changfa; Li, Jun; Wang, Chunsheng

    2014-01-01

    Thoracic endovascular aortic repair (TEVAR) is an effective strategy for type B dissection. Retrograde ascending dissection (RAD) intra-TEVAR is a rare complication on clinic. In this case, a 48-year-old Chinese man with Stanford type B aortic dissection suffered acute RAD during the TEVAR. And palliative stent grafts placement was performed in a local hospital, which earned the time for transfer and subsequent total arch replacement surgery in Zhongshan Hospital Fudan University. This report...

  4. Predictive factors related to low cardiac output syndrome following aortic valve replacement%主动脉瓣置换术后发生低心排出量综合征的预测因素探讨

    Institute of Scientific and Technical Information of China (English)

    马红; 张其霞; 李晓静; 郑晓燕

    2014-01-01

    Objective To investigate the predictive factors related to postoperative low cardiac output syndrome in pa-tients after aortic valve replacement due to aortic stenosis or regurgitation. Methods Three hundred patients with aortic valve defect due to aortic stenosis (AS, n=150) or aortic incompetence (AI, n=150), who underwent isolated aortic valve replacement were included in the study. Low cardiac output syndrome(LOS) was defined as the need for high dosages of inotropic medication, and/or intra- aortic bal oon pumping to sustain adequate hemodynamic status. Results Postoperative low cardiac output syn-drome developed in 86 patients (28.6%), including 39 patients with AS (26.0%) and 47 patients with AI (31.3%). The development of postoperative LOS were associated with the fol owing factors (odds ratio in parentheses): for AS group: advanced age (OR=4.7), obesity (OR=1.8), history of heart failure (OR=1.7), end- systolic (OR=5.5) and end- diastolic intraventricular septum thickness (OR=4.2), left atrial diameter (OR=1.6), mitral and tricuspid regurgitation (OR=1.9, 1.5) before surgery, LVEF≤50.0%(OR=5.4), left ventricular end systolic diameter (OR=1.7), left ventricular end diastolic diameter (OR=1.9) and mitral regurgitation (OR=4.1) in early postoperative period; for AI group:advanced age (OR=1.9), obesity (OR=4.8), history of heart failure (OR=1.7), LVEF≤50.0%(OR=1.8), left ventricular end- systolic (OR=4.5), end- diastolic diameters (OR=6.4), left ventricular end systolic di-ameter (OR=1.5), left ventricular end diastolic diameter(OR=1.6), and tricuspid regurgitation (OR=1.5) in preoperative period and left ventricular end- systolic (OR=4.7) and end- diastolic diameters (OR=6.1), and LVEF≤50.0% (OR=7.2) in early postoperative period. Conclusion The patients at high risk for the development of low cardiac output syndrome should be the focus of trials of new techniques of myocardial protection to effectively resuscitate the ischemic myocardium and

  5. Revisiting impressions using dual-arch trays.

    Science.gov (United States)

    Small, Bruce W

    2012-01-01

    Making routine perfect impressions is the goal of any restorative dentist. Using dual-arch trays is an easy, repeatable way to accomplish that goal, as long as each step is done before the next and each step is performed perfectly. This column reviewed several articles that support the metal dual-arch concept and provided some clinical tips that might help restorative dentists. The dual-arch technique does have its limits and is meant for one or two teeth in a quadrant when there are other teeth to occlude with. Also, if the case involves anterior guidance, a full-arch impression maybe advisable.

  6. Endovascular treatment of thoracic aortic diseases

    Directory of Open Access Journals (Sweden)

    Davidović Lazar

    2013-01-01

    Full Text Available Bacground/Aim. Endovascular treatment of thoracic aortic diseases is an adequate alternative to open surgery. This method was firstly performed in Serbia in 2004, while routine usage started in 2007. Aim of this study was to analyse initial experience in endovacular treatment of thoracic aortic diseses of three main vascular hospitals in Belgrade - Clinic for Vascular and Endovascular Surgery of the Clinical Center of Serbia, Clinic for Vascular Surgery of the Military Medical Academy, and Clinic for Vascular Surgery of the Institute for Cardiovascular Diseases “Dedinje”. Methods. Between March 2004. and November 2010. 41 patients were treated in these three hospitals due to different diseases of the thoracic aorta. A total of 21 patients had degenerative atherosclerotic aneurysm, 6 patients had penetrating aortic ulcer, 6 had posttraumatic aneurysm, 4 patients had ruptured thoracic aortic aneurysm, 1 had false anastomotic aneurysm after open repair, and 3 patients had dissected thoracic aneurysm of the thoracoabdominal aorta. In 15 cases the endovascular procedure was performed as a part of the hybrid procedure, after carotidsubclavian bypass in 4 patients and subclavian artery transposition in 1 patient due to the short aneurysmatic neck; in 2 patients iliac conduit was used due to hypoplastic or stenotic iliac artery; in 5 patients previous reconstruction of abdominal aorta was performed; in 1 patient complete debranching of the aortic arch, and in 2 patients visceral abdominal debranching were performed. Results. The intrahospital mortality rate (30 days was 7.26% (3 patients with ruptured thoracic aneurysms died. Endoleak type II in the first control exam was revealed in 3 patients (7. 26%. The patients were followed up in a period of 1-72 months, on average 29 months. The most devastating complication during a followup period was aortoesofageal fistula in 1 patient a year after the treatment of posttraumatic aneurysm. Conversion was

  7. Unicuspid aortic valve disease: a magnetic resonance imaging study

    Energy Technology Data Exchange (ETDEWEB)

    Debl, K.; Buchner, S.; Heinicke, N.; Riegger, G.; Luchner, A. [Klinik und Poliklinik fuer Innere Medizin II, Universitaetsklinikum Regensburg (Germany); Djavidani, B.; Poschenrieder, F.; Feuerbach, S. [Inst. fuer Roentgendiagnostik, Universitaetsklinikum Regensburg (Germany); Schmid, C.; Kobuch, R. [Klinik und Poliklinik fuer Herz-, Thorax- und herznahe Gefaesschirurgie, Universitaetsklinikum Regensburg (Germany)

    2008-11-15

    Purpose: congenitally malformed aortic valves are a common finding in adults with aortic valve disease. Most of these patients have bicuspid aortic valve disease. Unicuspid aortic valve disease (UAV) is rare. The aim of our study was to describe valve morphology and the dimensions of the proximal aorta in a cohort of 12 patients with UAV in comparison to tricuspid aortic valve disease (TAV) using magnetic resonance imaging (MRI). Materials and methods/results: MRI studies were performed on a 1.5 T scanner in a total of 288 consecutive patients with aortic valve disease. 12 aortic valves were retrospectively classified as UAV. Annulus areas and dimensions of the thoracic aorta were retrospectively compared to a cohort of 103 patients with TAV. In UAV, valve morphology was unicuspid unicommissural with a posterior commissure in all patients. Mean annulus areas and mean diameters of the ascending aorta were significantly greater in UAV compared to TAV (12.6 {+-} 4.7 cm{sup 2} vs. 8.7 {+-} 2.3 cm{sup 2}, p < 0.01 and 4.6 {+-} 0.7 cm vs. 3.6 {+-} 0.5 cm, p < 0.0001, respectively), while no differences were observed in the mean diameters of the aortic arch (2.3 {+-} 0.6 cm vs. 2.3 {+-} 0.4 cm, p = 0.69). The diameters of the descending aorta were slightly smaller in UAV compared to TAV (2.2 {+-} 0.5 cm vs. 2.6 {+-} 0.3 cm, p < 0.05). (orig.)

  8. Precision medical and surgical management for thoracic aortic aneurysms and acute aortic dissections based on the causative mutant gene.

    Science.gov (United States)

    Milewicz, Dianna; Hostetler, Ellen; Wallace, Stephanie; Mellor-Crummey, Lauren; Gong, Limin; Pannu, Hariyadarshi; Guo, Dong-chuan; Regalado, Ellen

    2016-04-01

    Almost one-quarter of patients presenting with thoracic aortic aneurysms (TAAs) or acute aortic dissections (TAADs) have an underlying mutation in a specific gene. A subset of these patients will have systemic syndromic features, for example, skeletal features in patients with Marfan Syndrome. It is important to note that the majority of patients with thoracic aortic disease will not have these syndromic features but many will have a family history of the disease. The genes predisposing to these thoracic aortic diseases are inherited in an autosomal dominant manner, and thirteen genes have been identified to date. As the clinical phenotype associated with each specific gene is defined, the data indicate that the underlying gene dictates associated syndromic features. More importantly, the underlying gene also dictates the aortic disease presentation, the risk for dissection at a given range of aortic diameters, the risk for additional vascular diseases and what specific vascular diseases occur associated with the gene. These results lead to the recommendation that the medical and surgical management of these patients be dictated by the underlying gene, and for patients with mutations in ACTA2, the specific mutation in the gene.

  9. Retalho de pericárdio pediculado vascularizado autógeno para aortoplastia e correção da coarctação simples de aorta torácica, ou associada à hipoplasia, atresia ou interrupção do arco aórtico Pediculated autologous vascularized pericardial flap aortoplasty for correction of simple aortic coarctation or associated with hypoplasia, atresia or interruption of aortic arch

    Directory of Open Access Journals (Sweden)

    Paulo Rodrigues da Silva

    2006-12-01

    . CONCLUSÃO: A técnica do emprego do retalho pericárdico pediculado, vascularizado e autógeno é a mais adequada e a mais completa em comparação com todas as outras técnicas cirúrgicas existentes para correção dos diferentes tipos de coarctação da aorta torácica, nas suas formas simples ou associadas a outras lesões aórticas. É indicada em todas as faixas etárias, inclusive em recém-natos.OBJECTIVE: Eighteen years ago, two young male patients of 8 months and 13 years with aortic coarctation associated to aortic hypoplasia between the left subclavian artery and the coarctated area, were submitted to surgical correction using a new world-pioneering surgical technique developed in our service. METHOD: This technique consists of sectioning the patent ductus arteriosus, followed by resection of all the coarctated tissue in the aortic wall and aortoplasty correction by means of the lengthwise implantation of a pediculated autogenous pericardial flap. This flap is inserted into the thoracic aorta, from the root of the left subclavian artery to 2.0 cm below the coarctated area. RESULTS: For both patients, the blood pressure and arterial pulses of both arms and legs have been normal since the surgery until the present moment. Clinical examination and Doppler evaluation evidenced no pressure gradient between arms and legs, normal blood flow and no pressure gradient through the coarctated area. Both patients were submitted to other evaluations 18 years after surgery, including cardiac and thoracic aortic catheterization followed by aortography. These evaluations demonstrated normal aortic configuration, with normal diameter, including the areas above and below the coarctated site. There was no evidence of any kind of degenerative lesions of the vascularized pericardial flap or re-coarctation of the lesion and no signs of aneurysms forming or the presence of atherosclerosis of the flap. Moreover, and very importantly, it was evident that the pediculated completely

  10. A quantitative method for defining high-arched palate using the Tcof1(+/-) mutant mouse as a model.

    Science.gov (United States)

    Conley, Zachary R; Hague, Molly; Kurosaka, Hiroshi; Dixon, Jill; Dixon, Michael J; Trainor, Paul A

    2016-07-15

    The palate functions as the roof of the mouth in mammals, separating the oral and nasal cavities. Its complex embryonic development and assembly poses unique susceptibilities to intrinsic and extrinsic disruptions. Such disruptions may cause failure of the developing palatal shelves to fuse along the midline resulting in a cleft. In other cases the palate may fuse at an arch, resulting in a vaulted oral cavity, termed high-arched palate. There are many models available for studying the pathogenesis of cleft palate but a relative paucity for high-arched palate. One condition exhibiting either cleft palate or high-arched palate is Treacher Collins syndrome, a congenital disorder characterized by numerous craniofacial anomalies. We quantitatively analyzed palatal perturbations in the Tcof1(+/-) mouse model of Treacher Collins syndrome, which phenocopies the condition in humans. We discovered that 46% of Tcof1(+/-) mutant embryos and new born pups exhibit either soft clefts or full clefts. In addition, 17% of Tcof1(+/-) mutants were found to exhibit high-arched palate, defined as two sigma above the corresponding wild-type population mean for height and angular based arch measurements. Furthermore, palatal shelf length and shelf width were decreased in all Tcof1(+/-) mutant embryos and pups compared to controls. Interestingly, these phenotypes were subsequently ameliorated through genetic inhibition of p53. The results of our study therefore provide a simple, reproducible and quantitative method for investigating models of high-arched palate.

  11. Image quality and diagnostic accuracy of unenhanced SSFP MR angiography compared with conventional contrast-enhanced MR angiography for the assessment of thoracic aortic diseases

    Energy Technology Data Exchange (ETDEWEB)

    Krishnam, Mayil S. [University of California, Cardiovascular and Thoracic Imaging, UCI Medical Center, Irvine, CA (United States); Tomasian, Anderanik; Malik, Sachin; Ruehm, Stefan G. [University of California at Los Angeles, Department of Radiological Sciences, Ronald Reagan Medical Center, Los Angeles, CA (United States); Desphande, Vibhas; Laub, Gerhard [Siemens Medical Solutions, Los Angeles, CA (United States)

    2010-06-15

    The purpose of this study was to determine the image quality and diagnostic accuracy of three-dimensional (3D) unenhanced steady state free precession (SSFP) magnetic resonance angiography (MRA) for the evaluation of thoracic aortic diseases. Fifty consecutive patients with known or suspected thoracic aortic disease underwent free-breathing ECG-gated unenhanced SSFP MRA with non-selective radiofrequency excitation and contrast-enhanced (CE) MRA of the thorax at 1.5 T. Two readers independently evaluated the two datasets for image quality in the aortic root, ascending aorta, aortic arch, descending aorta, and origins of supra-aortic arteries, and for abnormal findings. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were determined for both datasets. Sensitivity, specificity, and diagnostic accuracy of unenhanced SSFP MRA for the diagnosis of aortic abnormalities were determined. Abnormal aortic findings, including aneurysm (n = 47), coarctation (n = 14), dissection (n = 12), aortic graft (n = 6), intramural hematoma (n = 11), mural thrombus in the aortic arch (n = 1), and penetrating aortic ulcer (n = 9), were confidently detected on both datasets. Sensitivity, specificity, and diagnostic accuracy of SSFP MRA for the detection of aortic disease were 100% with CE-MRA serving as a reference standard. Image quality of the aortic root was significantly higher on SSFP MRA (P < 0.001) with no significant difference for other aortic segments (P > 0.05). SNR and CNR values were higher for all segments on SSFP MRA (P < 0.01). Our results suggest that free-breathing navigator-gated 3D SSFP MRA with non-selective radiofrequency excitation is a promising technique that provides high image quality and diagnostic accuracy for the assessment of thoracic aortic disease without the need for intravenous contrast material. (orig.)

  12. Tympanic ossicles and pharyngeal arches.

    Science.gov (United States)

    Whyte, J; Cisneros, A; Yus, C; Fraile, J; Obón, J; Vera, A

    2009-02-01

    We have performed a study on 11 human embryos regarding the development of the tympanic ossicles and their relationship with the first pharyngeal arch. After performing measurements to date the embryos and foetuses chronologically, we performed a meticulous dissection of the temporal bones. Subsequently, they were fixed in 10% formol, decalcified with 2% nitric acid, embedded in Paraplast, sectioned in 7-mm sequences and stained with Martin's trichrome technique. In the 21- and 24-mm cranium-raquis (CR) length human embryos, we have observed the head of the malleus and the body of the incus close to Meckel's cartilage, in addition to the handle of the malleus, the long limb of the incus and the stapes. Between them there was a mesenchymal band inside the primordium of the tympanic cavity. In the 27-mm CR embryo, the various components of the malleus and incus were fusing, and in the 30-mm CR embryo the union was complete. From our observations, we can conclude that the malleus and the incus are derived from the first and second pharyngeal arches.

  13. Flow in an Aortic Coarctation

    Science.gov (United States)

    Loma, Luis; Miller, Paul; Hertzberg, Jean

    2009-11-01

    Coarctation of the aorta is a congenital cardiovascular defect that causes a constriction in the descending thoracic aorta. To gain a better understanding of the cause of post-surgical problems, a rigid glass and a compliant in vitro model of the aortic arch and descending aorta with a coarctation were constructed. Near-physiologic compliance was obtained using a silicone elastomer. Stereoscopic PIV was used to obtain 3D velocity maps. Results show a high speed turbulent jet formed at the exit of the coarctation. Flow in the rigid model was significantly different from in the compliant model. In the rigid model, the jet was symmetric, creating a toroidal recirculation area. In the compliant model, the jet was directed towards the medial wall, inducing flow reversal only at the lateral wall. Peak velocities and turbulence intensities were higher in the rigid model, however shear rate values in the compliant model were significantly above both the rigid model and normal in vivo values at the medial wall. In both models the reattachment region fluctuated, creating oscillatory shear.

  14. Measurement of the aortic diameter in the asymptomatic Korean population: Assessment with multidetector CT

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang Hwan; Lee, Whal; Choi, Hyuck Jae; Kim, Dae Jin; Park, Eun Ah; Chung, Jin Wook; Park, Jae Hyung [Dept. of Radiology, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2013-08-15

    To determine normal reference values for aortic diameters in asymptomatic Korean adults. Three hundred adults without signs or symptoms of cardiovascular diseases were enrolled in this study. Aortic diameters were measured at nine predetermined levels on CT images. Aortic diameter measurements were adjusted for body surface area. Analysis of data was performed with regard to age, sex, weight, height and hypertension. Aortic diameters were 2.99 ± 0.57 cm at the ascending aorta, 2.54 ± 0.35 cm at the transverse aortic arch, 2.36 ± 0.35 cm at the proximal descending thoracic aorta (DTA), 2.23 ± 0.37 cm at the mid DTA, 2.17 ± 0.38 cm at the distal DTA, 2.16 ± 0.37 cm at the thoracoabdominal junction, 2.10, 00B1, 0.35 cm at the level of the celiac axis, 1.94, 00B1, 0.36 cm at the suprarenal aorta, 1.58 ± 0.24 cm at the aortic bifurcation. Men had slightly larger diameters than women (p < 0.05). All diameters increased with age and hypertension, with statistical significance (p < 0.01). And all aortic diameters increased with height (p < 0.05) except at the level of the aortic arch (p = 0.056), and increased with weight (p < 0.05) except at the level of the suprarenal aorta (p = 0.067). Male sex, higher weight and height, age and hypertension are associated with larger aortic diameters in asymptomatic Korean adults.

  15. Occlusal stability in shortened dental arches.

    Science.gov (United States)

    Witter, D J; Creugers, N H; Kreulen, C M; de Haan, A F

    2001-02-01

    Shortened dental arches consisting of anterior and premolar teeth have been shown to meet oral functional demands. However, the occlusal stability may be at risk as a result of tooth migration. The aim of this nine-year study was to investigate occlusal stability in shortened dental arches as a function over time. Occlusal stability indicators were: 'interdental spacing', 'occlusal contacts of anterior teeth in Intercuspal Position', 'overbite', 'occlusal tooth wear', and 'alveolar bone support'. Subjects with shortened dental arches (n = 74) were compared with subjects with complete dental arches (controls, n = 72). Repeated-measurement regression analyses were applied to assess age-dependent variables in the controls and to relate the occlusal changes to the period of time since the treatment that led to the shortened dental arches. Compared with complete dental arches, shortened dental arches had similar overbite and occlusal tooth wear. They showed more interdental spacing in the premolar regions, more anterior teeth in occlusal contact, and lower alveolar bone scores. Since the differences remained constant over time, we conclude that shortened dental arches can provide long-term occlusal stability. Occlusal changes were self-limiting, indicating a new occlusal equilibrium.

  16. Spontaneous retrograde dissection of the ascending aorta in a patient with a bicuspid aortic valve.

    Science.gov (United States)

    Akgullu, Cagdas; Hekim, Tolga; Eryilmaz, Ufuk; Kurtoğlu, Tünay; Gürcün, Uğur

    2013-06-23

    Bicuspid aortic valve (BAV) is a congenital anomaly associated with structural weakness of the aortic wall. Sudden onset of symptoms in patients with BAV, such as sudden severe back pain, and pulse inequality between the extremities or tension disparity should alert clinicians to acute aortic syndromes, as they require prompt diagnosis and management. Retrograde aortic dissection, which is a rare form of acute aortic syndrome, is an uncommon life-threatening entity and may produce atypical computed tomography (CT) or magnetic resonance imaging findings, leading to difficulty in diagnosis. We report on a 51-year-old male patient with BAV and spontaneous retrograde ascending aortic dissection. CT findings were confusing and the diagnosis was made via transoesophageal echocardiography. After the diagnosis, the patient was treated with a modified Bentall procedure. He did not have any complications and was stable four months after the operation.

  17. Aneurismas da aorta Aortic aneurysms

    Directory of Open Access Journals (Sweden)

    Januário M Souza

    1992-09-01

    Full Text Available Entre janeiro de 1979 e janeiro de 1992, foram realizadas 212 operações para correção de aneurismas e de dissecções da aorta. Neste trabalho serão analisados 104 procedimentos cirúrgicos (em 97 pacientes para correção de aneurismas. A idade dos pacientes variou de 14 a 79 anos (média 59,5 anos e o sexo predominante foi o masculino, com 75 pacientes. Os aneurismas localizavam-se na aorta ascendente em 46 pacientes, na croça em 8, na aorta descendente em 8, na aorta toráco-abdominal em 8, na aorta abdominal em 21, na aorta descendente e abdominal em 2, na aorta ascendente e tóraco-abdominal em 2, na aorta ascendente e descendente em 1, na aorta ascendente, croça e descendente em 1. Doenças cardiovasculares associadas estavam presentes em 39 pacientes, sendo valvopatia aórtica em 18 (excluídos os pacientes com ectasiaânulo-aórtíca, insuficiência coronária em 17, coarctação da aorta em 2, persistência do canal arterial em 1 e valvopatia mitral e aórtica em 1. A mortalidade imediata (hospitalar e/ou 30 dias foi de 14,4%, sendo de 27,7% (5/18 para pacientes com mais de 70 anos e de 11,3% (9/79 para pacientes com idade inferior a 70 anos. Os aneurismas localizados na aorta ascendente e croça foram operados como o auxílio de circulação extracorpórea. Parada circulatória e hipotermia profunda foram utilizadas em todos os pacientes com aneurisma da croça. O estudo tomográfico e angiográfico deve ser de toda a aorta, pela possibilidade de aneurismas de localizações múltiplas.Among 212 patients undergoing operation for aortic aneurysm and aortic dissection between January 1979 and January 1992, 97 were operated on for aneurysms. The aneurysms were localized in: ascending aorta in 46 patients, transverse aortic arch in 8, descending aorta in 8, thoracoabdominal aorta in 8, abdominal (infrarenal aorta in 21, descending and abdominal aorta in 2, ascending and thoracoabdominal aorta in 2, ascending and descending in 1

  18. Thoracic aortic aneurysm in infancy in aneurysms-osteoarthritis syndrome due to a novel SMAD3 mutation: further delineation of the phenotype

    NARCIS (Netherlands)

    Wischmeijer, A.; Laer, L. van; Tortora, G.; Bolar, N.A.; Camp, G. van; Fransen, E.; Peeters, N.; Bartolomeo, R. di; Pacini, D.; Gargiulo, G.; Turci, S.; Bonvicini, M.; Mariucci, E.; Lovato, L.; Brusori, S.; Ritelli, M.; Colombi, M.; Garavelli, L.; Seri, M.; Loeys, B.L.

    2013-01-01

    Recently, mutations in the SMAD3 gene were found to cause a new autosomal dominant aneurysm condition similar to Loeys-Dietz syndrome (LDS), mostly with osteoarthritis, called aneurysms-osteoarthritis syndrome (AOS). Our 3-year-old propositus underwent correction of an inguinal hernia at 3 months an

  19. [Late paraparesis as a postoperative complication in a patient undergoing the repair of a double aortic aneurysm].

    Science.gov (United States)

    Bonome González, C; Alvarez Refojo, F; Fernández Carballal, F; Rodríguez Alvarez, R

    1993-01-01

    We report a case of a fifty-seven (57)-years old man undergoing elective surgery of a thoracoabdominal and aortoiliac aneurysm in a single surgical time. The patients is operated undergoing general anesthetic combined with thoracic epidural blockade, and it was done two aortic cross-clamping: one to five cm of the aortic arch and the other to the infrarenal level. The most important intraoperative complications were during the thoracic aortic cross-clamping and the most important postoperative complication was related 48 hours later, to paraparesis after a hypotension episode what improved with rehabilitation treatment.

  20. Impact of Endografting on the Thoracic Aortic Anatomy: Comparative Analysis of the Aortic Geometry before and after the Endograft Implantation

    Energy Technology Data Exchange (ETDEWEB)

    Midulla, Marco, E-mail: marco.midulla@chru-lille.fr [University Hospital of Lille, Cardiovascular and Interventional Radiology (France); Moreno, Ramiro, E-mail: ramoroa@gmail.com [Rangueil University Hospital, Department of Radiology (France); Negre-Salvayre, Anne, E-mail: anne.negre-salvayre@inserm.fr [INSERM, UMR 1048, I2MC (France); Nicoud, Franc, E-mail: franck.nicoud@univ-montp2.fr [CNRS, UMR 5149 I3M, CC 051, University Montpellier II (France); Pruvo, Jean Pierre, E-mail: jean-pierre.pruvo@chru-lille.fr [University Hospital of Lille, Cardiovascular and Interventional Radiology (France); Haulon, Stephan, E-mail: stephan.haulon@chru-lille.fr [University Hospital of Lille, Department of Vascular Surgery (France); Rousseau, Hervé, E-mail: rousseau.h@chu-toulouse.fr [Rangueil University Hospital, Department of Radiology (France)

    2013-03-13

    PurposeAlthough the widespread acceptance of thoracic endovascular aortic repair (TEVAR) as a first-line treatment option for a multitude of thoracic aortic diseases, little is known about the consequences of the device implantation on the native aortic anatomy. We propose a comparative analysis of the pre- and postoperative geometry on a clinical series of patients and discuss the potential clinical implicationsMethodsCT pre- and postoperative acquisitions of 30 consecutive patients treated by TEVAR for different pathologies (20 thoracic aortic aneurysms, 6 false aneurysms, 3 penetrating ulcers, 1 traumatic rupture) were used to model the vascular geometry. Pre- and postoperative geometries were compared for each patient by pairing and matching the 3D models. An implantation site was identified, and focal differences were detected and described.ResultsSegmentation of the data sets was successfully performed for all 30 subjects. Geometry differences between the pre- and postoperative meshes were depicted in 23 patients (76 %). Modifications at the upper implantation site were detected in 14 patients (47 %), and among them, the implantation site involved the arch (Z0–3) in 11 (78 %).ConclusionModeling the vascular geometry on the basis of imaging data offers an effective tool to perform patient-specific analysis of the vascular geometry before and after the treatment. Future studies will evaluate the consequences of these changes on the aortic function.

  1. Management with the insufficient proximal landing zone for endovascular repair in aortic dissection

    Institute of Scientific and Technical Information of China (English)

    MA Hao; YANG Hong-yu; ZOU Jun-jie; ZHANG Xi-wei

    2011-01-01

    Background Sufficient length of the proximal landing zone (PLZ) is the key for a successful thoracic endovascular aortic repair (TEVAR) of an aortic lesion. The aim of this research was to investigate the safety, feasibility, efficacy, and problems of endovascular repair for aortic dissection with insufficient PLZ.Methods The clinical data between August 2005 and February 2010 from patients with insufficient PLZ for endovascular repair of aortic dissection were retrospectively reviewed. According to the classification proposed by Ishimaru, aortic zone 0 was involved in 3 cases, zone 1 in 10 cases, and zone 2 in 11 cases. A hybrid surgical procedure of supraortic debranching and revascularization, directly coverage the orifice of left subclavian artery, or a left common carotid artery chimney graft technique were performed to obtain an adequate proximal aortic landing zone.Results There was no significant difference in risk factors and diameter of the PLZ between Zone 0, Zone 1, and Zone 2. But the length of the PLZ was significantly different in the three groups (P <0.01). There was no significant difference in technical and clinical success rate between the groups.Conclusions The procedure for extending an insufficient PLZ for endovascular repair for aortic arch pathology is feasible and relatively safe. The applicability of TEVAR in such aortic disorders may be expanded.

  2. Aortic aneurysm and diverticulum of Kommerell: a dreadful concomitance

    Directory of Open Access Journals (Sweden)

    Fernando Peixoto Ferraz de Campos

    2012-12-01

    Full Text Available First described in 1936, the diverticulum of Kommerell (DOK is a dilatation of the proximal segment of an aberrant subclavian artery. Appearing more frequently in the left-sided aortic arch, the aberrant right subclavian artery passes behind the esophagus toward the right arm, causing symptoms in the minority of cases. Diagnosis is generally incidental with this pattern. When symptomatic, dysphagia, respiratory symptoms, hoarseness, chest pain, and upper limb ischemia are the most common complaints. Although debatable, the origin of DOK is accepted as being degenerative or congenital. The degenerative condition is normally associated with atherosclerosis and occurs more frequently after the age of 50 years with no gender predominance. Complications may be life threatening and are more commonly related to the diverticulum aneurysm or when associated with aortic diseases such as aneurysms or dissection. The authors present a case of a 67-year-old male with a history of acute chest pain, neurological disturbances, and hypertensive crisis. The diagnostic workup revealed an aortic arch aneurysm with intramural hematoma and a diverticulum aneurysm of Kommerell. Treatment was conservative at first. The patient presented a satisfactory outcome and was referred to an outpatient clinic for follow up and further therapeutic consolidation.

  3. Successful treatment for acute aortic dissection in pregnancy---bentall procedure concomitant with cesarean section

    Directory of Open Access Journals (Sweden)

    Xu Demin

    2011-10-01

    Full Text Available Abstract Acute aortic type A dissection is a life-threatening disease that requires immediate surgical intervention. When dissection occurs during pregnancy, it is of high risk for both the mother and the fetus. In this study, we reported two cases of acute aortic dissection in late pregnancy at 28 weeks and 32 weeks of gestation respectively. After the two patients underwent a cesarean section and delivered a baby, we performed composite graft replacement of the aortic valve, aortic root and ascending aorta, with re-implantation of the coronary arteries into the graft (Bentall procedure instead of repairing the arch with deep hypothermia and circulation arrest. Both mothers and children survived and recovered well.

  4. Extracorporeal membrane oxygenation in children with heart disease and down syndrome: a multicenter analysis.

    Science.gov (United States)

    Gupta, Punkaj; Gossett, Jeffrey M; Rycus, Peter T; Prodhan, Parthak

    2014-12-01

    The data on the outcomes of children with heart disease and Down syndrome receiving extracorporeal membrane oxygenation (ECMO) for cardiac or respiratory failure are limited. This study aimed to evaluate morbidity and mortality associated with ECMO in children with Down syndrome and heart disease. Children younger than 18 years undergoing heart surgery and ECMO reported in the Extracorporeal Life Support Organization (ELSO) registry (1998-2011) were included in the study. The registry was queried for the following five heart defects: common atrioventricular (AV) canal, tetralogy of Fallot, truncus arteriosus, transposition of great vessels, and interrupted aortic arch. Data collection included patient characteristics, ECMO characteristics, and outcomes. The outcomes evaluated included mortality, ECMO duration, and length of hospital stay for patients with Down syndrome and those with no Down syndrome. The study enrolled 2,815 patients qualified for inclusion. Of these patients, 121 had Down syndrome, whereas 2,694 had no genetic syndrome and were included in the control group. The median age of the patients was 45 days (interquartile range [IQR] 9-192 days), and the median weight was 3.8 kg (IQR 3.0-6.1 kg). The most common cardiac defects in Down syndrome group were common AV canal (63 %) and tetralogy of Fallot (40 %). The Down syndrome group included older patients with greater body weight than the control group. The mortality rate was lower in the Down syndrome group than in the control group (44 vs. 56 %; p = 0.01). The duration of ECMO and length of hospital stay were similar in the two groups. The findings showed that ECMO can be used for children with heart disease and Down syndrome with good results. The outcomes were comparable between the children with Down syndrome and the children without Down syndrome.

  5. Nonlinear Dynamics of Electrostatically Actuated MEMS Arches

    KAUST Repository

    Al Hennawi, Qais M.

    2015-05-01

    In this thesis, we present theoretical and experimental investigation into the nonlinear statics and dynamics of clamped-clamped in-plane MEMS arches when excited by an electrostatic force. Theoretically, we first solve the equation of motion using a multi- mode Galarkin Reduced Order Model (ROM). We investigate the static response of the arch experimentally where we show several jumps due to the snap-through instability. Experimentally, a case study of in-plane silicon micromachined arch is studied and its mechanical behavior is measured using optical techniques. We develop an algorithm to extract various parameters that are needed to model the arch, such as the induced axial force, the modulus of elasticity, and the initially induced initial rise. After that, we excite the arch by a DC electrostatic force superimposed to an AC harmonic load. A softening spring behavior is observed when the excitation is close to the first resonance frequency due to the quadratic nonlinearity coming from the arch geometry and the electrostatic force. Also, a hardening spring behavior is observed when the excitation is close to the third (second symmetric) resonance frequency due to the cubic nonlinearity coming from mid-plane stretching. Then, we excite the arch by an electric load of two AC frequency components, where we report a combination resonance of the summed type. Agreement is reported among the theoretical and experimental work.

  6. In-Plane Elastic Buckling of Arch

    Institute of Scientific and Technical Information of China (English)

    剧锦三; 郭彦林

    2002-01-01

    The in-plane elastic buckling behavior of arches is investigated using a new finite-element approach for the nonlinear analysis. The linear buckling, nonlinear primary buckling, and secondary bifurcation buckling behavior of arches are compared taking into account the large deformation and the effects of initial geometric imperfections or perturbations. The theoretical investigation emphasizes the nonlinear secondary bifurcation buckling behavior for a full span uniformly distributed load. The efficiency of compact method for tracing secondary buckling path is shown through several examples. Finally, a new structural design, which prevents the secondary bifurcation buckling by adding some crossed cables across the arch, is proposed to improve the limit load carrying capacity.

  7. Non-anatomical implantation of supra-aortic branches for customizing the standard form of Thoraflex™ hybrid prosthesis to a patient's anatomical requirements.

    Science.gov (United States)

    Urbanski, Paul P; Irimie, Vadim; Zembala, Michal

    2016-01-01

    Frozen elephant trunk technique represents a valuable therapeutic option for patients with extensive pathology of thoracic aorta. Thoraflex™ hybrid prosthesis (Vascutek, Terumo, Inchinnan, UK) is one of the commercially available devices in which a stent graft is combined with branched vascular tube mimicking the aortic arch anatomy. Owing to the sewing collar between the stented and unstented portions, this hybrid prosthesis offers a valuable addition to the surgical armamentarium for patients with huge thoracic aorta aneurysms, in whom the aortic diameter exceeds the diameter of available stent grafts. However, the standard factory form of the quadrifurcated arch prosthesis does not always fit to the patient's anatomy and demands a deviation from the anatomical anastomosing of the branches with the respective supra-aortic arteries. A technique of non-anatomical implantation of supra-aortic arteries for customizing the standard form of the Thoraflex prosthesis is described, which facilitates the surgery without limiting the functionality of this hybrid prosthesis.

  8. Determinant factors of Yemeni maxillary arch dimensions

    Directory of Open Access Journals (Sweden)

    Nabil Muhsen Al-Zubair

    2015-01-01

    Conclusion: Measurements of palatal depth and relationships of the canines to one another and to other teeth thus had the widest ranges, implying that these dimensions are the strongest determinants of maxillary arch size.

  9. 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.

  10. Fractionally Integrated Models With ARCH Errors

    OpenAIRE

    Hauser, Michael A.; Kunst, Robert M.

    1993-01-01

    Abstract: We introduce ARFIMA-ARCH models which simultaneously incorporate fractional differencing and conditional heteroskedasticity. We develop the likelihood function and a numerical estimation procedure for this model class. Two ARCH models - Engle- and Weiss-type - are explicitly treated and stationarity conditions are derived. Finite-sample properties of the estimation procedure are explored by Monte Carlo simulation. An application to the Standard & Poor 500 Index indicates existence o...

  11. Aortic Valve Disease

    Science.gov (United States)

    ... that is safest for you based on your individual symptoms and circumstances. Recovery Recovery Most aortic valve ... is safe to do so. You should anticipate spending several days, but likely not more than a ...

  12. Bicuspid aortic valve

    Science.gov (United States)

    ... aortic disease. In: Otto CM, Bonow RO, eds. Valvular Heart Disease: A Companion to Braunwald's Heart Disease . 4th ed. ... PA: Elsevier Saunders; 2014:chap 13. Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil ...

  13. Aortic Valve Regurgitation

    Science.gov (United States)

    ... inside your heart that involves heart valves. Rheumatic fever. Rheumatic fever — a complication of strep throat and once a ... the United States — can damage the aortic valve. Rheumatic fever is still prevalent in developing countries but rare ...

  14. Aortic Valve Stenosis

    Science.gov (United States)

    ... evaluation of aortic stenosis in adults. http://www.uptodate.com/home. Accessed April 29, 2014. Mohty D, ... Valvular heart disease in elderly adults. http://www.uptodate.com/home. Accessed May 2, 2014. Bonow RO, ...

  15. Stanford-A acute aortic dissection, inflammation, and metalloproteinases: a review.

    Science.gov (United States)

    Cifani, Noemi; Proietta, Maria; Tritapepe, Luigi; Di Gioia, Cira; Ferri, Livia; Taurino, Maurizio; Del Porto, Flavia

    2015-01-01

    Acute aortic dissection (AAD) is a life-threatening disease with an incidence of about 2.6-3.6 cases per 100,000/year. Depending on the site of rupture, AAD is classified as Stanford-A when the ascending aortic thoracic tract and/or the arch are involved, and Stanford-B when the descending thoracic aorta and/or aortic abdominal tract are targeted. It was recently shown that inflammatory pathways underlie aortic rupture in both type A and type B Stanford AAD. An immune infiltrate has been found within the middle and outer tunics of dissected aortic specimens. It has also been observed that the recall and activation of macrophages inside the middle tunic are key events in the early phases of AAD. Macrophages are able to release metalloproteinases (MMPs) and pro-inflammatory cytokines which, in turn, give rise to matrix degradation and neoangiogenesis. An imbalance between the production of MMPs and MMP tissue inhibitors is pivotal in the extracellular matrix degradation underlying aortic wall remodelling in dissections occurring both in inherited conditions and in atherosclerosis. Among MMPs, MMP-12 is considered a specific marker of aortic wall disease, whatever the genetic predisposition may be. The aim of this review is, therefore, to take a close look at the immune-inflammatory mechanisms underlying Stanford-A AAD.

  16. Endovascular treatment of a small infrarenal abdominal aortic aneurysm causing duodenal obstruction: Case report and literature review.

    Science.gov (United States)

    Esposito, Andrea; Menna, Danilo; Mansour, Wassim; Sirignano, Pasqualino; Capoccia, Laura; Speziale, Francesco

    2015-06-01

    Duodenal obstruction caused by abdominal aortic aneurysm was first described by Osler in 1905 and is named "aortoduodenal syndrome." This condition has always been treated by open surgical repair. We report the first case of aortoduodenal syndrome successfully treated by endovascular aneurysm repair. A 74-year-old male patient referred to our hospital complaining postprandial vomit, reporting a consistent weight loss in the latest weeks. Enhanced computed tomography scans showed a small saccular abdominal aortic aneurysm compressing duodenum and inferior vena cava without any other evident pathological finding. As the patient underwent a successful endovascular treatment of the abdominal aortic aneurysm, symptoms immediately resolved so that he started back to feed and progressively gained body weight. Despite aortoduodenal syndrome is generally caused by large abdominal aortic aneurysm, this condition has to be suspected also in case of small abdominal aortic aneurysm. Differently from what has been reported in literature, endovascular aneurysm repair could be effective in the treatment of aortoduodenal syndrome.

  17. Canadian Cardiovascular Society/Canadian Society of Cardiac Surgeons/Canadian Society for Vascular Surgery Joint Position Statement on Open and Endovascular Surgery for Thoracic Aortic Disease.

    Science.gov (United States)

    Appoo, Jehangir J; Bozinovski, John; Chu, Michael W A; El-Hamamsy, Ismail; Forbes, Thomas L; Moon, Michael; Ouzounian, Maral; Peterson, Mark D; Tittley, Jacques; Boodhwani, Munir

    2016-06-01

    In 2014, the Canadian Cardiovascular Society (CCS) published a position statement on the management of thoracic aortic disease addressing size thresholds for surgery, imaging modalities, medical therapy, and genetics. It did not address issues related to surgical intervention. This joint Position Statement on behalf of the CCS, Canadian Society of Cardiac Surgeons, and the Canadian Society for Vascular Surgery provides recommendations about thoracic aortic disease interventions, including: aortic valve repair, perfusion strategies for arch repair, extended arch hybrid reconstruction for acute type A dissection, endovascular management of arch and descending aortic aneurysms, and type B dissection. The position statement is constructed using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and has been approved by the primary panel, an international secondary panel, and the CCS Guidelines Committee. Advent of endovascular technology has improved aortic surgery safety and extended the indications of minimally invasive thoracic aortic surgery. The combination of safer open surgery with endovascular treatment has improved patient outcomes in this rapidly evolving subspecialty field of cardiovascular surgery.

  18. What Lies behind the Ischemic Stroke: Aortic Dissection?

    Directory of Open Access Journals (Sweden)

    Turgut Deniz

    2014-01-01

    Full Text Available Introduction. Some cases with aortic dissection (AD could present with various complaints other than pain, especially neurological and cardiovascular manifestations. AD involving the carotid arteries could be associated with many clinical presentations, ranging from stroke to nonspecific headache. Case Report. A 71-year-old woman was admitted to emergency department with vertigo which started within the previous one hour and progressed with deterioration of consciousness following speech disorder. On arrival, she was disoriented and uncooperative. Diffusion magnetic resonance imaging (MRI of brain was consistent with acute ischemia in the cerebral hemisphere. Fibrinolytic treatment has been planned since symptoms started within two hours. Echocardiography has shown the dilatation of ascending aorta with a suspicion of flap. Computed tomography (CT angiography has been applied and intimal flap has been detected which was consistent with aortic dissection, intramural hematoma of which was reaching from aortic arch to bilateral common carotid artery. Thereafter, treatment strategy has completely changed and surgical invention has been done. Conclusion. In patients who are admitted to the emergency department with the loss of consciousness and stroke, inadequacy of anamnesis and carotid artery involvement of aortic dissection should be kept in mind.

  19. Wall shear stress indicators in abnormal aortic geometries

    Science.gov (United States)

    Prahl Wittberg, Lisa; van Wyk, Stevin; Fuchs, Laszlo; Gutmark, Ephraim; Gutmark-Little, Iris

    2015-11-01

    Cardiovascular disease, such as atherosclerosis, occurs at specific locations in the arterial tree. Characterizing flow and forces at these locations is crucial to understanding the genesis of disease. Measures such as time average wall shear stress, oscillatory shear index, relative residence time and temporal wall shear stress gradients have been shown to identify plaque prone regions. The present paper examines these indices in three aortic geometries obtained from patients whose aortas are deformed due to a genetic pathology and compared to one normal geometry. This patient group is known to be prone to aortic dissection and our study aims to identify early indicators that will enable timely intervention. Data obtained from cardiac magnetic resonance imaging is used to reconstruct the aortic arch. The local unsteady flow characteristics are calculated, fully resolving the flow field throughout the entire cardiac cycle. The Quemada model is applied to account for the non-Newtonian properties of blood, an empirical model valid for different red blood cell loading. The impact of the deformed aortic geometries is analyzed to identify flow patterns that could lead to arterial disease at certain locations.

  20. [Inflammatory abdominal aortic aneurysm].

    Science.gov (United States)

    Ziaja, K; Sedlak, L; Urbanek, T; Kostyra, J; Ludyga, T

    2000-01-01

    The reported incidence of inflammatory abdominal aortic aneurysm (IAAA) is from 2% to 14% of patients with abdominal aortic aneurysm and the etiology of this disease is still discussed--according to the literature several pathogenic theories have been proposed. From 1992 to 1997 32 patients with IAAA were operated on. The patients were mostly symptomatic--abdominal pain was present in 68.75% cases, back pain in 31.25%, fever in 12.5% and weight loss in 6.25% of the operated patients. In all the patients ultrasound examination was performed, in 4 patients CT and in 3 cases urography. All the patients were operated on and characteristic signs of inflammatory abdominal aortic aneurysm like: thickened aortic wall, perianeurysmal infiltration or retroperitoneal fibrosis with involvement of retroperitoneal structures were found. In all cases surgery was performed using transperitoneal approach; in three cases intraoperatively contiguous abdominal organs were injured, which was connected with their involvement into periaortic inflammation. In 4 cases clamping of the aorta was done at the level of the diaphragmatic hiatus. 3 patients (9.37%) died (one patient with ruptured abdominal aortic aneurysm). Authors present diagnostic procedures and the differences in the surgical tactic, emphasizing the necessity of the surgical therapy in patients with inflammatory abdominal aortic aneurysm.

  1. Transvenous digital subtraction angiography (DSA) of the thoracic aorta after surgical correction of aortic coarctation

    Energy Technology Data Exchange (ETDEWEB)

    Arlart, I.P.; Hamann, H.; Stenz, R.

    1985-01-01

    25 patients (normotensive n = 19, hypertensive n = 6) underwent DSA control following corrective surgery of a coarcatation of the thoracic aorta to exclude vascular complications. Simultaneously pressure gradients were determined between upper and lower extremities using the doppler-sonography. DSA was diagnostic in all patients. 2 cases showed a patch-dependent dilatation, in 1 case an anastomotic aneurysm could be demonstrated. In 6 patients with hypoplastic aortic arch and 2 patients with a slight reduction of the diameter in the anastomotic region a pathological pressure-gradient was measured. In these patients the ratio of aortic arch rsp. anastomosis/descending aorta was reduced in diameter (< 0,66) and area (< 44). Hypertension could not be related to pressure gradients or vascular disorders.

  2. Histopathological study of congenital aortic valve malformations in 32 children

    Institute of Scientific and Technical Information of China (English)

    HUANG Ping; WANG Hongwei; LI Yanping; CHENG Peixuan; LIU Qingjun; ZHANG Zhenlu; LIU Jianying

    2007-01-01

    The histopathological characteristics of congenital aortic valve malformations in children were investigated.All the native surgically excised aortic valves from 32 pediatric patients suffering from symptomatic aortic valve dysfunction due to congenital aortic valve malformations between January 2003 and December 2005 were studied macroscopically and microscopically.The patients' medical records were reviewed and the clinical information was extracted.The diagnosis was made by the clinical presentation,preoperative echocardiography,intraoperative examination,and postoperative histopathological study,excluding rheumatic ot degenerative aortic valve diseases,infective endocarditis and primary connective tissue disorders,e.g.Marfan syndrome.Among 32 children with congenital aortic valve malformations,the age was ranged from six to 18 years,with a mean of 14.9 years,and there were 27 boys and five girls (male:female = 5.4:1).There were five cases of aortic stenosis (AS,15.62%),25 cases of aortic insufficiency (AI,78.13 %)and two cases of AS-AI (6.25%),without other valve diseases.Twenty cases still had other congenital heart diseases:ventricular septal defect (19 cases),patent ductus arteriosus (two cases),double-chambered right ventricle (one case),aneurysm of the right anterior aortic sinus of valsalva (three cases).Histopathological examination indicated that the cusps became thickening with unequal size,irregular shape (coiling and prolapse edge),enhanced hardness,and partly calcification.Microscopic investigation revealed the unsharp structure of valve tissue,fibrosis,myxomatous,reduced collagen fiber,rupture of elastic fibers,different degrees of infiltration of inflammatory cells,secondary calcareous and lipid deposit,and secondary fibrosis.Congenital aortic valve malformations in children involve males more than females,mostly associated with other congenital heart diseases.Aortic insufficiency is more common in children with congenital aortic valve

  3. Automatic aorta segmentation and valve landmark detection in C-arm CT: application to aortic valve implantation.

    Science.gov (United States)

    Zheng, Yefeng; John, Matthias; Liao, Rui; Boese, Jan; Kirschstein, Uwe; Georgescu, Bogdan; Zhou, S Kevin; Kempfert, Jörg; Walther, Thomas; Brockmann, Gernot; Comaniciu, Dorin

    2010-01-01

    C-arm CT is an emerging imaging technique in transcatheter aortic valve implantation (TAVI) surgery. Automatic aorta segmentation and valve landmark detection in a C-arm CT volume has important applications in TAVI by providing valuable 3D measurements for surgery planning. Overlaying 3D segmentation onto 2D real time fluoroscopic images also provides critical visual guidance during the surgery. In this paper, we present a part-based aorta segmentation approach, which can handle aorta structure variation in case that the aortic arch and descending aorta are missing in the volume. The whole aorta model is split into four parts: aortic root, ascending aorta, aortic arch, and descending aorta. Discriminative learning is applied to train a detector for each part separately to exploit the rich domain knowledge embedded in an expert-annotated dataset. Eight important aortic valve landmarks (three aortic hinge points, three commissure points, and two coronary ostia) are also detected automatically in our system. Under the guidance of the detected landmarks, the physicians can deploy the prosthetic valve properly. Our approach is robust under variations of contrast agent. Taking about 1.4 seconds to process one volume, it is also computationally efficient.

  4. Current role of endovascular therapy in Marfan patients with previous aortic surgery

    Directory of Open Access Journals (Sweden)

    Ibrahim Akin

    2008-02-01

    Full Text Available Ibrahim Akin, Stephan Kische, Tim C Rehders, Tushar Chatterjee, Henrik Schneider, Thomas Körber, Christoph A Nienaber, Hüseyin InceDepartment of Medicine, Division of Cardiology at the University Hospital Rostock, Rostock School of Medicine, Ernst-Heydemann-Str. 6, 18057 Rostock, GermanyAbstract: The Marfan syndrome is a heritable disorder of the connective tissue which affects the cardiovascular, ocular, and skeletal system. The cardiovascular manifestation with aortic root dilatation, aortic valve regurgitation, and aortic dissection has a prevalence of 60% to 90% and determines the premature death of these patients. Thirty-four percent of the patients with Marfan syndrome will have serious cardiovascular complications requiring surgery in the first 10 years after diagnosis. Before aortic surgery became available, the majority of the patients died by the age of 32 years. Introduction in the aortic surgery techniques caused an increase of the 10 year survival rate up to 97%. The purpose of this article is to give an overview about the feasibility and outcome of stent-graft placement in the descending thoracic aorta in Marfan patients with previous aortic surgery.Keywords: Marfan syndrome, aortic dissection, root replacement, stent-graft, previous aortic surgery

  5. Arch Index: An Easier Approach for Arch Height (A Regression Analysis

    Directory of Open Access Journals (Sweden)

    Hironmoy Roy

    2012-04-01

    Full Text Available Background: Arch-height estimation though practiced usually in supine posture; is neither correct nor scientific as referred in literature, which favour for standing x-rays or arch-index as yardstick. In fact the standing x-rays can be excused for being troublesome in busy OPD, but an ink-footprint on simple graph-sheet can be documented, as it is easier, cheaper and requires almost no machineries and expertisation. Objective: So this study aimed to redefine the inter-relationship of the radiological standing arch-heights with the arch-index for correlation and regression so that from the later we can derive the radiographical standing arch-height values indirectly, avoiding the actual maneuver. Methods: The study involved 103 adult subjects attending at a tertiary care hospital of North Bengal. From the standing x-rays of foot, the standing navicular, talar heights were measured, and ‘normalised’ with the foot length. In parallel foot-prints also been obtained for arch-index. Finally variables analysed by SPSS software. Result: The arch-index showed significant negative correlations and simple linear regressions with standing navicular height, standing talar height as well as standing normalised navicular and talar heights analysed in both sexes separately with supporting mathematical equations. Conclusion: To measure the standing arch-height in a busy OPD, it is wise to have the foot-print first. Arch-index once get known, can be put in the equations as derived here, to predict the preferred standing arch-heights in either sex.

  6. Intramural aortic hematomas; Intramurale Haematome der Aorta

    Energy Technology Data Exchange (ETDEWEB)

    Raab, B.W.; Vosshenrich, R.; Fischer, U.; Funke, M.; Grabbe, E. [Goettingen Univ. (Germany). Abt. fuer Roentgendiagnostik

    2001-08-01

    Intramural hematomas (IMH) are regarded as a hemorrhage into the aortic wall. In general a traumatic form can be differentiated from a spontaneous non-traumatic. There is a predisposition of IMH with arterial hypertension and mesoectodermal dysplastic syndromes. The diagnosis is established with the clinical presentation in combination with the findings of different imaging modalities. Acute and subacute discomfort associated with a tendency of collaps are considered as typical presenting complaints. A semicircular or concentric thickening of the aortic wall with the absence of blood flow or a dissection membran are typical findings in transesophageal echocardiography, computed tomography and magnetic resonance tomography. From a differential diagnostic point of view a distinction from atherosclerotic wall changes, intraluminal thrombi and inflammatory aortic diseases is essential. The IMH is considered as an early presentation of aortic dissection, put into and treated according to the Stanford classification. In the course of natural history an IMH can disappear, rupture or progress into a classic aortic dissection. (orig.) [German] Das intramurale Haematom (IMH) ist eine Einblutung in die Aortenwand. Grundsaetzlich kann zwischen traumatischer Genese und einer atraumatischen spontanen Form unterschieden werden. Praedisponierend sind arterielle Hypertension und mesoektodermale Dysplasiesyndrome. Die Diagnose ergibt sich aus der Klinik und den Befunden in den bildgebenden Verfahren. Zu den klinischen Symptomen zaehlen akut oder subakut auftretende Schmerzen, assoziiert mit einer Kollapsneigung. Typische Befunde in der transoesophagealen Echokardiographie, der Computer- oder der Magnetresonanztomographie sind eine halbmondfoermige oder konzentrische Wandverdickung der Aorta ohne Nachweis von Blutfluss oder einer Dissektionsmembran mit Dichtewerten bzw. Signalintensitaeten in Abhaengigkeit vom Alter der Einblutung. Differenzialdiagnostisch ist eine Abgrenzung

  7. Pregnancy and Thoracic Aortic Disease: Managing the Risks.

    Science.gov (United States)

    Wanga, Shaynah; Silversides, Candice; Dore, Annie; de Waard, Vivian; Mulder, Barbara

    2016-01-01

    The most common aortopathies in women of childbearing age are bicuspid aortic valve, coarctation of the aorta, Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome, SMAD3 aortopathy, Turner syndrome, and familial thoracic aneurysm and dissection. The hemodynamic and hormonal changes of pregnancy increase the risk of progressive dilatation or dissection of the aorta in these women. The presence of hypertension increases the risk further. Therefore, appropriate preconception counselling is advised. For women who become pregnant, serial follow-up by a specialized multidisciplinary team throughout pregnancy and postpartum period is required. In this review we discuss risk assessment and management strategies for women with aortopathies.

  8. Screening for Abdominal Aortic Aneurysm

    Science.gov (United States)

    Understanding Task Force Recommendations Screening for Abdominal Aortic Aneurysm The U.S. Preventive Services Task Force (Task Force) has issued a final recommendation statement on Screening for Abdominal Aortic Aneurysm. This final ...

  9. Abdominal aortic aneurysm repair - open

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/007392.htm Abdominal aortic aneurysm repair - open To use the sharing features on this page, please enable JavaScript. Open abdominal aortic aneurysm repair is surgery to fix a widened part ...

  10. Tubular Steel Arch Stabilized by Textile Membranes

    Directory of Open Access Journals (Sweden)

    Ondrej Svoboda

    2016-10-01

    Full Text Available Tubular steel arch supporting textile membrane roofing is investigated experimentally and numerically. The stabilization effects of the textile membrane on in-plane and out-of-plane behavior of the arch is of primary interest. First a model of a large membrane structure tested in laboratory is described. Prestressed membranes of PVC coated polyester fabric Ferrari® Précontraint 702S were used as a currently standard and excellent material. The test arrangement, loading and resulting load/deflection values are presented. The supporting structure consisted of two steel arch tubes, outer at edge of the membrane and inner supporting interior of the membrane roofing. The stability and strength behavior of the inner tube under both symmetrical and asymmetrical loading was monitored and is shown in some details. Second the SOFiSTiK software was employed to analyze the structural behavior in 3D, using geometrically nonlinear analysis with imperfections (GNIA. The numerical analysis, FE mesh sensitivity, the membrane prestressing and common boundary conditions are validated by test results. Finally a parametrical study concerning stability of mid arch with various geometries in a membrane structure with several supporting arches is presented, with recommendations for a practical design.

  11. Dental Arch Dimension of Malay Ethnic Group

    Directory of Open Access Journals (Sweden)

    H. A. Mohammad

    2011-01-01

    Full Text Available Problem statement: Many previous studies tried to define and put specific measurements for dental arches dimension in different ethnics groups. However, these studies may be specific to an ethnic group and cannot always be applied to other ethnic types. The aim of this study: is to obtain specific dental arch dimensions for Malaysian Malay ethnic groups and compare between both genders. Approach: Involved clinical examination, collection and analysis of 60 dental cast of Malaysian Malay subjects from pure ethnic group (30 males and 30 females, 20-24 years old. All dental landmarks were located and determined and subsequently measurements had been done according to specific points have been selected. Statistical analysis were done using t-test. Results: Dental arch dimension were established in normal occlusion for Malaysian Malay ethnic group in this study. No significant difference was observed in arch dimensions between male and female of Malaysian Malay. Conclusion: These measurement should be considered during treatment, especially in prosthodontics and orthodontics where arch dimension can be modified appreciably.

  12. [Congenital aortic stenosis].

    Science.gov (United States)

    Yamaguchi, M

    2001-08-01

    Recent advances in and controversies concerning the management of children with congenital valvular aortic stenosis are discussed. In neonates with critical aortic stenosis, improved survival has recently been reported after surgical open valvotomy and balloon valvuloplasty, although it is difficult at this point to compare the results of the two procedures and determine their differential indications. Good results have also been achieved after extended aortic valvuloplasty for recurrent aortic stenosis and/or insufficiency, but the length of follow-up in these patients is still short. The technique first reported in 1991 for bilateral enlargement fo a small annulus permits the insertion of an aortic valve 3-4 sizes larger than the native annulus. It entails no risk of distorting the mitral valve, damaging the conduction system or important branches of the coronary arteries, or resulting in left ventricular dysfunction. The Ross procedure is now widely applied in the West, with reports of early mortality rates of less than 5% and event-free survival rates of 80-90% during follow-up of 4-8 years. Longer follow-up and continued careful evaluation are required to resolve the issue of possible dilatation and subsequent neoaortic valve dysfunction and pulmonary stenosis due to allograft degeneration after pulmonary autograft root replacement in children.

  13. Nonsyndromic bilateral multiple retained primary incisors in mandibular arch: Rare case report

    Directory of Open Access Journals (Sweden)

    D Deepa

    2016-01-01

    Full Text Available A disturbed eruption process creates a clinical situation that is challenging to diagnose and treat. The clinical arena of tooth eruption disorders includes both syndromic and nonsyndromic problems varying from delayed eruption to a complete failure of eruption or sometimes retained primary teeth. This paper reports a rare case of retained bilateral primary incisors on mandibular arch in a systemically healthy patient with eruption of the permanent central and lateral incisors bilaterally.

  14. [An inflammatory aortic aneurysm ruptured into the retroperitoneum and an extensive communication of the aneurysm with the vena cava inferior].

    Science.gov (United States)

    Tovar Martín, E; Acea Nebril, B; Díaz Pardeiro, P

    1993-01-01

    Aortocaval fistula is a rare complication of abdominal aortic aneurysms that occurs with a frequency of 1% of operative cases or less. In this report we present a case of aortocaval fistula associated with ruptured and inflammatory aortic aneurysm that became apparent after evacuation of the thrombus. The inferior cava was ligated. We discuss the clinical syndrome and the management of patients with aortocaval fistula secondary to an abdominal aortic aneurysm and the results of surgical repair.

  15. No beneficial effect of general and specific anti-inflammatory therapies on aortic dilatation in Marfan mice.

    Directory of Open Access Journals (Sweden)

    Romy Franken

    Full Text Available AIMS: Patients with Marfan syndrome have an increased risk of life-threatening aortic complications, mostly preceded by aortic dilatation. In the FBN1(C1039G/+ Marfan mouse model, losartan decreases aortic root dilatation. We recently confirmed this beneficial effect of losartan in adult patients with Marfan syndrome. The straightforward translation of this mouse model to man is reassuring to test novel treatment strategies. A number of studies have shown signs of inflammation in aortic tissue of Marfan patients. This study examined the efficacy of anti-inflammatory therapies in attenuating aortic root dilation in Marfan syndrome and compared effects to the main preventative agent, losartan. METHODS AND RESULTS: To inhibit inflammation in FBN1(C1039G/+ Marfan mice, we treated the mice with losartan (angiotensin II receptor type 1 inhibitor, methylprednisolone (corticosteroid or abatacept (T-cell-specific inhibitor. Treatment was initiated in adult Marfan mice with already existing aortic root dilatation, and applied for eight weeks. Methylprednisolone- or abatacept-treated mice did not reveal a reduction in aortic root dilatation. In this short time frame, losartan was the only treatment that significantly reduced aorta inflammation, transforming growth factor-beta (TGF-β signaling and aortic root dilatation rate in these adult Marfan mice. Moreover, the methylprednisolone-treated mice had significantly more aortic alcian blue staining as a marker for aortic damage. CONCLUSION: Anti-inflammatory agents do not reduce the aortic dilatation rate in Marfan mice, but possibly increase aortic damage. Currently, the most promising therapeutic drug in Marfan syndrome is losartan, by blocking the angiotensin II receptor type 1 and thereby inhibiting pSmad2 signaling.

  16. Surgical treatment of Stanford type A aortic dissection%Stanford A型主动脉夹层的外科治疗

    Institute of Scientific and Technical Information of China (English)

    孙永辉; 王安彪; 邹承伟; 李德才; 李红昕; 王正军; 訾捷; 张文龙; 张海洲; 范全心

    2011-01-01

    , including ascending aorta replacement only ( 10 cases), ascending aorta replacement + arch or semi-arch replacement ( 13 cases), ascending aorta replacement + arch or semi-arch replacement with stented elephant trunk (27 cases), ascending aorta replacement + four branches covered stent graft implantation (7 cases ) and Bentall operation only (8 cases). Concomitant procedures were coronary artery bypass grafting (4 cases), aortic valvuloplasty( 12 cases), aortic valve replacement( 8 cases ) or Bentall operation (6 cases). Deep hypothermic circulation arrest with selective cerebral perfusion was employed in cases of aortic arch replacement and/or endovascular stent repair. Results In the operations, the CPB, clamp and circulation arrest time were 86 -350 ( 188.3 ±60.9), 60-248 (101.1 ±40.0) and 20-120 (53.9 ± 24.3 ) minutes, respectively. There were two deaths early after operation. One died from low cardiac output syndrome and the other died from renal failure. Postoperative intensive care stay was 1 ~ 63 (4.2 ±8.8) days and the hospital stay was 13 ~ 106 (24.8 ±13.8) days. Complications consisted of motor aphasia (n = 1 ), cerebral embolism( n = 1 ), pulmonary infection ( n = 3 ), renal failure ( n = 1 ), hemorrhage ( n = 5 ), mental disorder( n = 4), tracheotomy ( n = 1 ), and voice hoarseness ( n = 1 ). Conclusion Aortic dissection is the most common catastrophic event that involves the aorta, however, the prospects of early surgical treatment are satisfying. The key points are quick, correct diagnosis and proper surgical approaches.

  17. Aortic valve replacement

    DEFF Research Database (Denmark)

    Kapetanakis, Emmanouil I; Athanasiou, Thanos; Mestres, Carlos A;

    2008-01-01

    mortality were collected. Group analysis by patient geographic distribution and by annular diameter of the prosthesis utilized was conducted. Patients with a manufacturer's labeled prosthesis size > or = 21 mm were assigned to the 'large' aortic size subset, while those with a prosthesis size ... assigned to the 'small' aortic size subset. Effective orifice area indices were calculated for all patients to assess the geographic distribution of patient-prosthesis mismatch. Univariable and multivariable logistic regression analyses adjusting for possible confounding variables were performed. RESULTS...... differences in the distribution of either gender or BSA. In the multivariable model, south European patients were seven times more likely to receive a smaller-sized aortic valve (OR = 6.5, 95% CI = 4.82-8.83, p

  18. Robotic aortic surgery.

    Science.gov (United States)

    Duran, Cassidy; Kashef, Elika; El-Sayed, Hosam F; Bismuth, Jean

    2011-01-01

    Surgical robotics was first utilized to facilitate neurosurgical biopsies in 1985, and it has since found application in orthopedics, urology, gynecology, and cardiothoracic, general, and vascular surgery. Surgical assistance systems provide intelligent, versatile tools that augment the physician's ability to treat patients by eliminating hand tremor and enabling dexterous operation inside the patient's body. Surgical robotics systems have enabled surgeons to treat otherwise untreatable conditions while also reducing morbidity and error rates, shortening operative times, reducing radiation exposure, and improving overall workflow. These capabilities have begun to be realized in two important realms of aortic vascular surgery, namely, flexible robotics for exclusion of complex aortic aneurysms using branched endografts, and robot-assisted laparoscopic aortic surgery for occlusive and aneurysmal disease.

  19. The association between dental arch dimensions and occurrence of Finnish dental consonant misarticulations in cleft lip/palate children.

    Science.gov (United States)

    Laitinen, J; Ranta, R; Pulkkinen, J; Haapanen, M L

    1998-10-01

    The aim of this study was to examine whether maxillary and mandibular dental arch width, length, and palatal height dimensions are associated with the occurrence of misarticulations (phonetic or phonologic errors) in the dental consonants /r/, /s/, and /1/ in different cleft types and sexes. The subjects were 263 (109 girls, 154 boys) 6-year-old Finnish-speaking non-syndromic children with isolated cleft palate (CP, n=79), deft lip/alveolus (CL(A), n=77), unilateral (UCLP, n=80), and bilateral (BCLP, n=27) cleft lip and palate. Dental plaster casts were measured by two authors using the technique of Moorrees, and auditive speech was analyzed with high reliability by two speech pathologists. The results showed that the occurrence of misarticulations increased and dental arch dimensions decreased with the severity of the cleft. Narrower and shorter maxillary arches as well as shallower palates were related to problems with the studied dental consonants. Mandibular arch dimensions were not related to the misarticulations. However, statistical analysis did not reveal significant differences in dental arch dimensions between subjects with and without misarticulations when they were compared separately for different cleft types. The etiology of clefting per se--isolated deft palate versus cleft lip with or without deft palate--did not seem to explain the associations between dental arch dimensions and the studied misarticulations.

  20. Aortic outflow cannula tip design and orientation impacts cerebral perfusion during pediatric cardiopulmonary bypass procedures.

    Science.gov (United States)

    Menon, Prahlad G; Antaki, James F; Undar, Akif; Pekkan, Kerem

    2013-12-01

    Poor perfusion of the aortic arch is a suspected cause for peri- and post-operative neurological complications associated with cardiopulmonary bypass (CPB). High-speed jets from 8 to 10FR pediatric/neonatal cannulae delivering ~1 L/min of blood can accrue sub-lethal hemolytic damage while also subjecting the aorta to non-physiologic flow conditions that compromise cerebral perfusion. Therefore, we emphasize the importance of cannulation strategy and hypothesize engineering better CPB perfusion through a redesigned aortic cannula tip. This study employs computational fluid dynamics to investigate novel diffuser-tipped aortic cannulae for shape sensitivity to cerebral perfusion, in an in silico cross-clamped aortic arch model modeled with fixed outflow resistances. 17 parametrically altered configurations of an 8FR end-hole and several diffuser cone angled tips in combination with jet incidence angles toward or away from the head-neck vessels were studied. Experimental pressure-flow characterizations were also conducted on these cannula tip designs. An 8FR end-hole aortic cannula delivering 1 L/min along the transverse aortic arch was found to give rise to backflow from the brachicephalic artery (BCA), irrespective of angular orientation, for the chosen ascending aortic insertion location. Parametric alteration of the cannula tip to include a diffuser cone angle (tested up to 7°) eliminated BCA backflow for any tested angle of jet incidence. Experiments revealed that a 1 cm long 10° diffuser cone tip demonstrated the best pressure-flow performance improvement in contrast with either an end-hole tip or diffuser cone angles greater than 10°. Performance further improved when the diffuser was preceded by an expanded four-lobe swirl inducer attachment-a novel component. In conclusion, aortic cannula orientation is crucial in determining net head-neck perfusion but precise angulations and insertion-depths are difficult to achieve practically. Altering the cannula tip

  1. 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.

  2. The murine angiotensin II-induced abdominal aortic aneurysm model: rupture risk and inflammatory progression patterns

    Directory of Open Access Journals (Sweden)

    Richard Y Cao

    2010-07-01

    Full Text Available An abdominal aortic aneurysm (AAA is an enlargement of the greatest artery in the body defined as an increase in diameter of 1.5-fold. AAAs are common in the elderly population and thousands die each year from their complications. The most commonly used mouse model to study the pathogenesis of AAA is the angiotensin II (Ang II infusion method delivered via osmotic mini-pump for 28 days. Here, we studied the site-specificity and onset of aortic rupture, characterized three-dimensional (3D images and flow patterns in developing AAAs by ultrasound imaging, and examined macrophage infiltration in the Ang II model using 65 apolipoprotein E deficient mice. Aortic rupture occurred in 16 mice (25 % and was nearly as prevalent at the aortic arch (44 % as it was in the suprarenal region (56 % and was most common within the first seven days after Ang II infusion (12 of 16; 75 %. Longitudinal ultrasound screening was found to correlate nicely with histological analysis and AAA volume renderings showed a significant relationship with AAA severity index. Aortic dissection preceded altered flow patterns and macrophage infiltration was a prominent characteristic of developing AAAs. Targeting the inflammatory component of AAA disease with novel therapeutics will hopefully lead to new strategies to attenuate aneurysm growth and aortic rupture.

  3. Iatrogenic neonatal type B aortic dissection: comprehensive MRI-based diagnosis and follow-up

    Energy Technology Data Exchange (ETDEWEB)

    Geiger, Julia; Markl, M. [University Hospital Freiburg, Department of Diagnostic Radiology and Medical Physics, Freiburg (Germany); Stiller, B.; Arnold, R. [University Hospital Freiburg, Department of Congenital Heart Disease and Pediatric Cardiology, Freiburg (Germany); Schlensak, C. [University Hospital Freiburg, Department of Cardiovascular Surgery, Freiburg (Germany)

    2011-10-15

    Neonatal aortic dissection is rare and most frequently iatrogenic. Decision making and appropriate imaging are highly challenging for pediatric cardiologists and radiologists. We present MRI and echocardiographic findings in the follow-up at 6 months of age of a boy with a conservatively treated iatrogenic neonatal aortic dissection (type B). To evaluate the morphology of the aortic arch and descending aorta, we carried out multidirectional time-resolved three-dimensional flow-analysis and contrast-enhanced MR angiography (CE-MRA). The MRI and Doppler echocardiographic results were closely comparable. Three-dimensional visualization helped assess details of blood flow acceleration and alteration caused by the dissection, and played a key role in our deciding not to treat surgically. (orig.)

  4. Effects of psychiatric disorders on Type A acute aortic dissection pathogenesis and analysis of follow-up results

    Directory of Open Access Journals (Sweden)

    Paolo Nardi

    2015-12-01

    Full Text Available Aims: A connection between psychiatric disorders (PDs and Type A acute aortic dissection (AAD has not been shown. The aim of this study was to define the psychological profile of patients treated for AAD, and to analyze the prevalence of PDs in their medical histories, in the immediate postoperative period, and at a mid-term follow-up. Patients and Methods: From March 2005 to October 2014, 240 consecutive patients underwent surgery for AAD. 60 patients (mean age 60+/-13 years; 43 males underwent psychiatric consultation postoperatively, and they represent the subjects of our retrospective study. Ascending aorta +/- arch replacement was performed in 43 patients, whereas the Bentall procedure +/- arch replacement was performed in 17. Data were retrospectively analyzed. Follow-ups were completed in 59 patients (mean duration 35+/-23 months. Results: PDs were present in the medical histories of 34 patients. Postoperatively, in 28 cases, a definitive diagnosis of PD (group PD was made in agreement with the diagnostic and statistical manual of mental disorders-IV criteria, including: Major depression (n=13, anxious-depressive syndrome (n=6, bipolar disorder Type 2 (n=4, panic attacks (n=2, paranoid schizophrenia (n=1, and anxiety (n=2. 32 patients without a definitive psychiatric diagnosis were classified as Group non-PD. In the postoperative period, clinical manifestations of PDs, including delirium, persistent spatio-temporal disorientation, and psychomotor agitation were evident in 22 patients (78% in group PD versus 8 patients (25% in group non-PD (P<0.0001. During follow-up, only one death for non-cardiac reasons occurred in group PD. There were no suicides; only 10 patients of group PD required PD treatment (P<0.0001 vs. early postoperative findings; 4 patients in group non-PD required PD treatment. Conclusion: Our findings suggest a strong relationship between PD and AAD. Because the psychiatric conditions appeared to be largely stable after

  5. MAT2A mutations predispose individuals to thoracic aortic aneurysms.

    Science.gov (United States)

    Guo, Dong-chuan; Gong, Limin; Regalado, Ellen S; Santos-Cortez, Regie L; Zhao, Ren; Cai, Bo; Veeraraghavan, Sudha; Prakash, Siddharth K; Johnson, Ralph J; Muilenburg, Ann; Willing, Marcia; Jondeau, Guillaume; Boileau, Catherine; Pannu, Hariyadarshi; Moran, Rocio; Debacker, Julie; Bamshad, Michael J; Shendure, Jay; Nickerson, Deborah A; Leal, Suzanne M; Raman, C S; Swindell, Eric C; Milewicz, Dianna M

    2015-01-08

    Up to 20% of individuals who have thoracic aortic aneurysms or acute aortic dissections but who do not have syndromic features have a family history of thoracic aortic disease. Significant genetic heterogeneity is established for this familial condition. Whole-genome linkage analysis and exome sequencing of distant relatives from a large family with autosomal-dominant inheritance of thoracic aortic aneurysms variably associated with the bicuspid aortic valve was used for identification of additional genes predisposing individuals to this condition. A rare variant, c.1031A>C (p.Glu344Ala), was identified in MAT2A, which encodes methionine adenosyltransferase II alpha (MAT IIα). This variant segregated with disease in the family, and Sanger sequencing of DNA from affected probands from unrelated families with thoracic aortic disease identified another MAT2A rare variant, c.1067G>A (p.Arg356His). Evidence that these variants predispose individuals to thoracic aortic aneurysms and dissections includes the following: there is a paucity of rare variants in MAT2A in the population; amino acids Glu344 and Arg356 are conserved from humans to zebrafish; and substitutions of these amino acids in MAT Iα are found in individuals with hypermethioninemia. Structural analysis suggested that p.Glu344Ala and p.Arg356His disrupt MAT IIα enzyme function. Knockdown of mat2aa in zebrafish via morpholino oligomers disrupted cardiovascular development. Co-transfected wild-type human MAT2A mRNA rescued defects of zebrafish cardiovascular development at significantly higher levels than mRNA edited to express either the Glu344 or Arg356 mutants, providing further evidence that the p.Glu344Ala and p.Arg356His substitutions impair MAT IIα function. The data presented here support the conclusion that rare genetic variants in MAT2A predispose individuals to thoracic aortic disease.

  6. Automatic aorta segmentation and valve landmark detection in C-arm CT for transcatheter aortic valve implantation.

    Science.gov (United States)

    Zheng, Yefeng; John, Matthias; Liao, Rui; Nöttling, Alois; Boese, Jan; Kempfert, Jörg; Walther, Thomas; Brockmann, Gernot; Comaniciu, Dorin

    2012-12-01

    Transcatheter aortic valve implantation (TAVI) is a minimally invasive procedure to treat severe aortic valve stenosis. As an emerging imaging technique, C-arm computed tomography (CT) plays a more and more important role in TAVI on both pre-operative surgical planning (e.g., providing 3-D valve measurements) and intra-operative guidance (e.g., determining a proper C-arm angulation). Automatic aorta segmentation and aortic valve landmark detection in a C-arm CT volume facilitate the seamless integration of C-arm CT into the TAVI workflow and improve the patient care. In this paper, we present a part-based aorta segmentation approach, which can handle structural variation of the aorta in case that the aortic arch and descending aorta are missing in the volume. The whole aorta model is split into four parts: aortic root, ascending aorta, aortic arch, and descending aorta. Discriminative learning is applied to train a detector for each part separately to exploit the rich domain knowledge embedded in an expert-annotated dataset. Eight important aortic valve landmarks (three hinges, three commissures, and two coronary ostia) are also detected automatically with an efficient hierarchical approach. Our approach is robust under all kinds of variations observed in a real clinical setting, including changes in the field-of-view, contrast agent injection, scan timing, and aortic valve regurgitation. Taking about 1.1 s to process a volume, it is also computationally efficient. Under the guidance of the automatically extracted patient-specific aorta model, the physicians can properly determine the C-arm angulation and deploy the prosthetic valve. Promising outcomes have been achieved in real clinical applications.

  7. Precast Pearl-Chain concrete arch bridges

    DEFF Research Database (Denmark)

    Halding, Philip Skov; Hertz, Kristian Dahl; Schmidt, Jacob Wittrup

    2015-01-01

    A Pearl-Chain Bridge is a closed-spandrel arch bridge consisting of a number of straight pre-fabricated so called Super-Light Deck elements put together in an arch shape by post-tensioning cables. Several Pearl-Chain arches can be positioned adjacent to each other by a crane to achieve a bridge...... the technology was used. We also study other important components and details in the Pearl-Chain Bridge concept and review the effects of different types of loads. A theoretical case study of a circular 30 m span Pearl-Chain Bridge is presented showing the influence of a number of parameters: The number of post-tensioning......, and gives a large positive bending moment below the load and a smaller negative bending moment in the unloaded side. When the Pearl-Chain Bridge concept is compared to other pre-fabricated arch bridge solutions we find a number of advantages when using Pearl-Chain Bridges: Straight elements, combination...

  8. Unoperated aortic aneurysm

    DEFF Research Database (Denmark)

    Perko, M J; Nørgaard, M; Herzog, T M;

    1995-01-01

    From 1984 to 1993, 1,053 patients were admitted with aortic aneurysm (AA) and 170 (15%) were not operated on. The most frequent reason for nonoperative management was presumed technical inoperability. Survivals for patients with thoracic, thoracoabdominal, and abdominal AA were comparable...... hypertension were predictive of mortality, whereas...

  9. Dilatation of the aneurysmal sac after total arch replacement.

    Science.gov (United States)

    Watanuki, Hirotaka; Ogino, Hitoshi; Matsuda, Hitoshi; Minatoya, Kenji; Sasaki, Hiroaki; Fukuda, Tetsuya; Kitamura, Soichiro

    2008-02-01

    In our institution, total arch replacement for distal arch aneurysms is performed through a median sternotomy with antegrade selective cerebral perfusion. The distal anastomosis to the completely transected descending aorta is made through the aneurysmal sac. We report on three interesting cases presenting late dilatation of the aneurysmal sac due to collateral flow after total arch replacement.

  10. Assembly and lifting of Pearl-Chain arches

    DEFF Research Database (Denmark)

    Halding, Philip Skov; Hertz, Kristian Dahl; Viebæk, N.E.

    2015-01-01

    Pearl-Chain arches were invented at the Technical University of Denmark in cooporation with the company Abeo A/S. The system uses specially designed, pre-fabricated concrete elements that are post-tensioned together into an arch shape, which is then lifted into place. The arches can be used both...

  11. Aortic valve replacement for Libman-Sacks endocarditis

    Science.gov (United States)

    Keenan, Jack B; Janardhanan, Rajesh; Larsen, Brandon T; Khalpey, Zain

    2016-01-01

    A 24-year-old man with systemic lupus erythematosus and antiphospholipid syndrome complicated by lupus nephritis presented with acute limb ischaemia secondary to an embolus. Following embolectomy, the patient underwent a transthoracic echocardiogram which revealed a large vegetation on all three cusps of the aortic valve. The patient was taken for an urgent aortic valve replacement with a mechanical valve. Cultures of one cusp remained sterile. Histopathological examination of the remaining two cusps revealed sterile fibrin-rich thrombotic vegetations characteristic of non-bacterial thrombotic endocarditis. PMID:27702929

  12. Acute aortic dissection: be aware of misdiagnosis

    Directory of Open Access Journals (Sweden)

    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.

  13. Involvement of the thymus and cellular immune system in craniofacial malformation syndromes.

    Science.gov (United States)

    Scheuerle, A E; Good, R A; Habal, M B

    1990-04-01

    Craniofacial structures, the aortic arch, thymus, and parathyroid glands all arise from the embryologic pharyngeal pouches, and DiGeorge and Job craniofacial malformation syndromes have defined immunologic deficiencies. The question addressed by this study is whether patients with other pharyngeal pouch malformations could also have immunologic abnormalities. Twelve patients, 4 female and 8 male, were selected at random from the Tampa Bay Craniofacial Center. Their diagnoses included: cleft lip/cleft palate, hemifacial microsomia/Goldenhar syndrome, Treacher-Collins syndrome, craniofacial hemangiomata, cranio-synostosis syndromes, and Tessier 13 cleft. Fresh blood samples were analyzed against age-matched controls for immunoglobin number, using immunoelectrophoresis, T-cell, B-cell, and natural killer cell quantity via Coulter counter and monoclonal antibody labeling, as well as lymphocyte stimulation and response functions with phytohemagglutinin, concanavalin A, pokeweed mitogen, and Staphylococcus aureus mitogens. All patients studied had some abnormality of their immune systems. Seven had specific T-cell abnormalities and three patients had abnormalities in all three categories studied. This indicates that patients with any pharyngeal pouch malformation may have an abnormality of the immune system.

  14. Palliative stent graft placement combined with subsequent open surgery for retrograde ascending dissection intra-thoracic endovascular aortic repair.

    Science.gov (United States)

    Zhu, Kai; Lai, Hao; Guo, Changfa; Li, Jun; Wang, Chunsheng

    2014-12-01

    Thoracic endovascular aortic repair (TEVAR) is an effective strategy for type B dissection. Retrograde ascending dissection (RAD) intra-TEVAR is a rare complication on clinic. In this case, a 48-year-old Chinese man with Stanford type B aortic dissection suffered acute RAD during the TEVAR. And palliative stent grafts placement was performed in a local hospital, which earned the time for transfer and subsequent total arch replacement surgery in Zhongshan Hospital Fudan University. This report suggests that the palliative strategy may be an option for RAD in some specific situation.

  15. Open triple-branched stent graft applied to patient of acute type a aortic dissection with Aberrant Right Subclavian Artery

    OpenAIRE

    Guo, Changfa; Zhu, Kai; Xu, Demin; Wang, Chunsheng

    2013-01-01

    A 57-year-old Chinese male patient presented with Standford type A aortic dissection with an aberrant right subclavian artery (ARSA). At operation, the ascending aorta was replaced by a mono–branch vascular prosthesis with the branch bypassing to the ARSA; the triple-branched stent graft was inserted into the true lumen of the arch and proximal descending aorta (covering the origin of the ARSA) with each sidearm graft being positioned into the aortic branches; and then its proximal end was su...

  16. Palliative stent graft placement combined with subsequent open surgery for retrograde ascending dissection intra-thoracic endovascular aortic repair

    Science.gov (United States)

    Zhu, Kai; Guo, Changfa; Li, Jun

    2014-01-01

    Thoracic endovascular aortic repair (TEVAR) is an effective strategy for type B dissection. Retrograde ascending dissection (RAD) intra-TEVAR is a rare complication on clinic. In this case, a 48-year-old Chinese man with Stanford type B aortic dissection suffered acute RAD during the TEVAR. And palliative stent grafts placement was performed in a local hospital, which earned the time for transfer and subsequent total arch replacement surgery in Zhongshan Hospital Fudan University. This report suggests that the palliative strategy may be an option for RAD in some specific situation. PMID:25590002

  17. Post-stenotic aortic dilatation

    Directory of Open Access Journals (Sweden)

    Jahangiri Marjan

    2006-03-01

    Full Text Available Abstract Aortic stenosis is the most common valvular heart disease affecting up to 4% of the elderly population. It can be associated with dilatation of the ascending aorta and subsequent dissection. Post-stenotic dilatation is seen in patients with AS and/or aortic regurgitation, patients with a haemodynamically normal bicuspid aortic valve and following aortic valve replacement. Controversy exists as to whether to replace the aortic root and ascending aorta at the time of aortic valve replacement, an operation that potentially carries a higher morbidity and mortality. The aetiology of post-stenotic aortic dilatation remains controversial. It may be due to haemodynamic factors caused by a stenotic valve, involving high velocity and turbulent flow downstream of the stenosis, or due to intrinsic pathology of the aortic wall. This may involve an abnormality in the process of extracellular matrix remodelling in the aortic wall including inadequate synthesis, degradation and transport of extracellular matrix proteins. This article reviews the aetiology, pathology and management of patients with post-stenotic aortic dilatation.

  18. Familial Thoracic Aortic Aneurysm with Dissection Presenting as Flash Pulmonary Edema in a 26-Year-Old Man

    Directory of Open Access Journals (Sweden)

    Sabry Omar

    2014-01-01

    Full Text Available We are reporting a case of familial thoracic aortic aneurysm and dissection in a 26-year-old man with no significant past medical history and a family history of dissecting aortic aneurysm in his mother at the age of 40. The patient presented with cough, shortness of breath, and chest pain. Chest X-ray showed bilateral pulmonary infiltrates. CT scan of the chest showed a dissection of the ascending aorta. The patient underwent aortic dissection repair and three months later he returned to our hospital with new complaints of back pain. CT angiography showed a new aortic dissection extending from the left carotid artery through the bifurcation and into the iliac arteries. The patient underwent replacement of the aortic root, ascending aorta, total aortic arch, and aortic valve. The patient recovered well postoperatively. Genetic studies of the patient and his children revealed no mutations in ACTA2, TGFBR1, TGFBR2, TGFB2, MYH11, MYLK, SMAD3, or FBN1. This case report focuses on a patient with familial TAAD and discusses the associated genetic loci and available screening methods. It is important to recognize potential cases of familial TAAD and understand the available screening methods since early diagnosis allows appropriate management of risk factors and treatment when necessary.

  19. 78 FR 79300 - Cardiovascular Devices; Reclassification of Intra-Aortic Balloon and Control Systems for Acute...

    Science.gov (United States)

    2013-12-30

    ... Intra-Aortic Balloon and Control Systems for Acute Coronary Syndrome, Cardiac and Non- Cardiac Surgery... coronary syndrome, cardiac and non- cardiac surgery, or complications of heart failure, a preamendments..., Drug, and Cosmetic Act (the FD&C Act), as amended by the Medical Device Amendments of 1976 (the...

  20. Natural history and outcome of patients with intramural hematomas and penetrating aortic ulcers.

    Science.gov (United States)

    Mousa, Albeir Y; Bozzay, Joseph; AbuRahma, Ali F

    2015-06-01

    All pathologies of acute aortic syndromes should be precisely diagnosed for prompt therapy. Intramural hematomas, as well as penetrating ulcers can be encountered in these patients. Presentations, clinical scenarios, and proper management are outlined in this review, which sums up available current literature to provide the vascular specialist with an adequate understanding of these unique syndromes.

  1. MRI evaluation of the aortic disease; Zastosowanie tomografii rezonansu magnetycznego w rozpoznawaniu chorob aorty

    Energy Technology Data Exchange (ETDEWEB)

    Kapuscinski, O.; Polkowski, J. [Zaklad Radiologii, Inst. Kardiologii, Warsaw-Anin (Poland); Zaleska, T. [Oddzial Kardiologii, CSK, Warsaw-Miedzylesie (Poland); Walecki, J.; Biesiadko, M. [Zaklad Diagnostyki Obrazowej, CMKP and CSK, Warsaw-Miedzylesie (Poland)

    1994-12-31

    The goal of our study was to establish the value of MRI in diagnosing aortic disease. During 3 years period 46 patients were examined (12 women aged from 6 to 62 years and 34 men aged from 8 to 72 years). In 28 cases the thoracic aorta was examined, in 6 cases the abdominal aorta and in 12 - both the thoracic and the abdominal aorta. MR images were performed on MRT 50 A Toshiba unit 0.5 T. MR images demonstrated aortic aneurysm (caused by atherosclerosis or trauma), aortic dissections, aortic anomalies (i.e. in Turner syndrome) and non-specific aortitis and vasculitis. We regard MRI as preferable noninvasive imaging technique in diagnosing aortic disease. (author) 13 refs, 3 figs

  2. In-plane elastic stability of fixed parabolic shallow arches

    Institute of Scientific and Technical Information of China (English)

    CAI JianGuo; FENG Jian; CHEN Yao; HUANG LiFeng

    2009-01-01

    The nonlinear behavior of fixed parabolic shallow arches subjected to a vertical uniform load is inves-tigated to evaluate the in-plane buckling load. The virtual work principle method is used to establish the non-linear equilibrium and buckling equations. Analytical solutions for the non-linear in-plane sym-metric snap-through and antisymmetric bifurcation buckling loads are obtained. Based on the least square method, an approximation for the symmetric buckling load of fixed parabolic arch is proposedto simplify the solution process. And the relation between modified slenderness and buckling modes are discussed. Comparisons with the results of finite element analysis demonstrate that the solutions are accurate. A cable-arch structure is presented to improve the in-plane stability of parabolic arches. The comparison of buckling loads between cable-arch systems and arches only show that the effect of cables becomes more evident with the increase of arch's modified slenderness.

  3. Thoracic aortic aneurysm

    Science.gov (United States)

    ... age Connective tissue disorders such as Marfan or Ehlers-Danlos syndrome Inflammation of the aorta Injury from falls ... of connective tissue disorders (such as Marfan or Ehlers-Danlos syndrome) Chest or back discomfort

  4. Tobacco smoking and aortic aneurysm

    DEFF Research Database (Denmark)

    Sode, Birgitte F; Nordestgaard, Børge G; Grønbæk, Morten

    2012-01-01

    General Population Study, respectively. According to the magnitude of the hazard ratios, tobacco consumption was the most important risk factor for hospitalization and death from aortic aneurysm, followed by male sex and hypertension in both cohorts. The population attributable risk of aortic aneurysm...... outcomes due to tobacco consumption was 64% and 47% in the Copenhagen City Heart Study and Copenhagen General Population Study, respectively, and ranked highest among population attributable risks of aortic aneurysm in both cohorts. The absolute 10-year risk for hospitalization or death from aortic......BACKGROUND: We determined the predictive power of tobacco smoking on aortic aneurysm as opposed to other risk factors in the general population. METHODS: We recorded tobacco smoking and other risk factors at baseline, and assessed hospitalization and death from aortic aneurysm in 15,072 individuals...

  5. Evolution Characteristic Analysis of Pressure-arch of a Double-arch Tunnel in Water-rich Strata

    Directory of Open Access Journals (Sweden)

    C. L. Li

    2016-03-01

    Full Text Available It is of importance to analyze the morphological characterization, the evolution process and the skewed effect of pressure-arch of a double-arch tunnel in the water-rich strata. Taking a buried depth 80 m double-arch tunnel as an example, a computational model of the double-arch tunnel was built by using FLAC3D technique. Then considering some aspects including groundwater conditions, tunnel depth, construction sequences and permeability coefficients, the coupling effect of stress field and seepage field in the pressure-arch of the double-arch tunnel was analyzed. The results show that the thickness of the pressure-arch induced by step-by-step excavation and display a step-descent skewed distribution from the left to the right of the double-arch tunnel. The permeability coefficient has a significant influence on the shape and the skewed effect of the pressure arch. The excavation of the bench method has a better arching condition than that of the expanding method. The abtained results provide a basic reference for the rock reinforcement design and safety construction of double-arch tunnels in the water-rich strata.

  6. Ruptured abdominal aortic aneurysm.

    Science.gov (United States)

    Sachs, T; Schermerhorn, M

    2010-06-01

    Ruptured abdominal aortic aneurysm (AAA) continues to be one of the most lethal vascular pathologies we encounter. Its management demands prompt and efficient evaluation and repair. Open repair has traditionally been the mainstay of treatment. However, the introduction of endovascular techniques has altered the treatment algorithm for ruptured AAA in most major medical centers. We present recent literature and techniques for ruptured AAA and its surgical management.

  7. Infrarenal Aortic Occlusion

    Science.gov (United States)

    Traverso, L. W.; Baker, J. D.; Dainko, E. A.; Machleder, H. I.

    1978-01-01

    Twenty-eight patients with total occlusion of the infrarenal aorta have been seen at the UCLA Hospitals in the past 11 years. Claudication was the presenting complaint in all but one patient, with one-third having ischemic rest pain. The average age of these patients was 54 years, and their histories revealed a surprising absence of myocardial infarction, stroke, or diabetes, although 40% had essential hypertension. Heavy tobacco use, however, was characteristic of the entire group. Arteriography proved valuable in identifying and characterizing the vascular abnormalities, but posed problems in technique and interpretation. Significant distal arterial disease was detected radiographically in only 21% of these patients. Operative correction of the aortic occlusion was performed on 26 patients, 18 by aortic bypass grafts and eight by aorto-iliac endarterectomy, with one early postoperative death. Although the thrombus extended to the renal artery origins in 77% of the cases, a well-designed technical approach did not require renal artery occlusion. Using serial creatinine determinations, one case of renal insufficiency was detected which was associated with prolonged postoperative hypotension. Although the extent of distal disease was more severe in those who underwent bypass, symptoms of claudication returned earlier and were more prominent in the endarterectomy group. This recurrence of systems was not favorably altered by sympathectomy performed concomitantly with the initial procedure. Even though this condition seems to pose difficult technical obstacles and has a poor prognosis, infrarenal aortic occlusion can be successfully treated by aortic bypass, with favorable long-term results, if particular attention is paid to elements of the preoperative evaluation and the intraoperative technical requirements peculiar to this relatively uncommon disease entity. ImagesFig. 1.Fig. 2.Fig. 3. PMID:646479

  8. Micromanaging Abdominal Aortic Aneurysms

    Directory of Open Access Journals (Sweden)

    Lars Maegdefessel

    2013-07-01

    Full Text Available The contribution of abdominal aortic aneurysm (AAA disease to human morbidity and mortality has increased in the aging, industrialized world. In response, extraordinary efforts have been launched to determine the molecular and pathophysiological characteristics of the diseased aorta. This work aims to develop novel diagnostic and therapeutic strategies to limit AAA expansion and, ultimately, rupture. Contributions from multiple research groups have uncovered a complex transcriptional and post-transcriptional regulatory milieu, which is believed to be essential for maintaining aortic vascular homeostasis. Recently, novel small noncoding RNAs, called microRNAs, have been identified as important transcriptional and post-transcriptional inhibitors of gene expression. MicroRNAs are thought to “fine tune” the translational output of their target messenger RNAs (mRNAs by promoting mRNA degradation or inhibiting translation. With the discovery that microRNAs act as powerful regulators in the context of a wide variety of diseases, it is only logical that microRNAs be thoroughly explored as potential therapeutic entities. This current review summarizes interesting findings regarding the intriguing roles and benefits of microRNA expression modulation during AAA initiation and propagation. These studies utilize disease-relevant murine models, as well as human tissue from patients undergoing surgical aortic aneurysm repair. Furthermore, we critically examine future therapeutic strategies with regard to their clinical and translational feasibility.

  9. Systemic Inflammatory Response and Severe Thrombocytopenia after Endovascular Thoracic Aortic Aneurysm Repair

    Directory of Open Access Journals (Sweden)

    Valentina Silvestrin

    2017-01-01

    Full Text Available After Endovascular repair of thoracic aortic aneurysm, a systemic inflammatory response, named postimplantation syndrome, can develop. This syndrome is characterized by fever, leukocytosis, and elevated CRP plasma levels and its pathogenetic mechanisms are still unknown. Although this syndrome generally resolves within few days, some patients develop a persisting severe inflammatory reaction leading to mild or severe complications. Here we describe the case of a male patient who developed postimplantation inflammatory syndrome and severe thrombocytopenia after endovascular repair of thoracic aortic aneurysm. Treatment with prednisone (50 mg/bid for two weeks did not improve the clinical and laboratory findings. We utilized danazol, a weak androgen that has been shown to be effective in the treatment of immune and idiopathic thrombocytopenic purpura, and after 12 days of treatment with danazol (200 mg/bid, the patient improved progressively and platelet number increased up to 53,000/μL. Patients undergoing endovascular repair of thoracic aortic aneurysm should be carefully monitored for the development of postimplantation syndrome. This clinical condition is relatively common after the endovascular repair of aortic aneurysm but is rarely observed after endovascular repair of thoracic aortic aneurysms. The different known therapeutical approaches are still empiric, with reported beneficial effects with the use of NSAID, corticosteroids, and danazol.

  10. Systemic Inflammatory Response and Severe Thrombocytopenia after Endovascular Thoracic Aortic Aneurysm Repair

    Science.gov (United States)

    Silvestrin, Valentina; Bonvini, Stefano; Antonello, Michele; Grego, Franco; Vettor, Roberto

    2017-01-01

    After Endovascular repair of thoracic aortic aneurysm, a systemic inflammatory response, named postimplantation syndrome, can develop. This syndrome is characterized by fever, leukocytosis, and elevated CRP plasma levels and its pathogenetic mechanisms are still unknown. Although this syndrome generally resolves within few days, some patients develop a persisting severe inflammatory reaction leading to mild or severe complications. Here we describe the case of a male patient who developed postimplantation inflammatory syndrome and severe thrombocytopenia after endovascular repair of thoracic aortic aneurysm. Treatment with prednisone (50 mg/bid) for two weeks did not improve the clinical and laboratory findings. We utilized danazol, a weak androgen that has been shown to be effective in the treatment of immune and idiopathic thrombocytopenic purpura, and after 12 days of treatment with danazol (200 mg/bid), the patient improved progressively and platelet number increased up to 53,000/μL. Patients undergoing endovascular repair of thoracic aortic aneurysm should be carefully monitored for the development of postimplantation syndrome. This clinical condition is relatively common after the endovascular repair of aortic aneurysm but is rarely observed after endovascular repair of thoracic aortic aneurysms. The different known therapeutical approaches are still empiric, with reported beneficial effects with the use of NSAID, corticosteroids, and danazol. PMID:28154580

  11. Hemolytic anemia after operation for aortic dissection using teflon felt strips.

    Science.gov (United States)

    Nakamura, Yuki; Ogino, Hitoshi; Matsuda, Hitoshi; Minatoya, Kenji; Sasaki, Hiroaki; Kitamura, Soichiro

    2008-05-01

    We report three cases of hemolytic anemia caused by anastomotic stenosis after surgical treatment for aortic dissection in which internal and external Teflon (DuPont, Wilmington, DE) felt strips were used for reinforcement of the aortic stump. To detect this complication, laboratory findings typical of red cell fragmentation syndrome as well as appropriate imaging modalities are necessary. As a precaution, it is necessary to be meticulous when stitching the internal felt strip.

  12. 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.

  13. Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Disease

    DEFF Research Database (Denmark)

    Mylotte, Darren; Lefevre, Thierry; Søndergaard, Lars

    2014-01-01

    BACKGROUND: Limited information exists describing the results of transcatheter aortic valve (TAV) replacement in patients with bicuspid aortic valve (BAV) disease (TAV-in-BAV). OBJECTIVES: This study sought to evaluate clinical outcomes of a large cohort of patients undergoing TAV-in-BAV. METHODS...

  14. Aortic root geometry in aortic stenosis patients (a SEAS substudy)

    DEFF Research Database (Denmark)

    Bahlmann, Edda; Nienaber, Christoph A; Cramariuc, Dana

    2011-01-01

    AIMS: To report aortic root geometry by echocardiography in a large population of healthy, asymptomatic aortic stenosis (AS) patients in relation to current vendor-specified requirements for transcatheter aortic valve implantation (TAVI). METHODS AND RESULTS: Baseline data in 1481 patients...... with asymptomatic AS (mean age 67 years, 39% women) in the Simvastatin Ezetimibe in AS study were used. The inner aortic diameter was measured at four levels: annulus, sinus of Valsalva, sinotubular junction and supracoronary, and sinus height as the annulo-junctional distance. Analyses were based on vendor......-specified requirements for the aortic root geometry for current available prostheses, CoreValve and Edwards-Sapien. The ratio of sinus of Valsalva height to sinus width was 1:2. In multivariate linear regression analysis, larger sinus of Valsalva height was associated with older age, larger sinus of Valsalva diameter...

  15. [Thoracic aortic dissection revealed by systemic cholesterol embolism].

    Science.gov (United States)

    Braem, L; Paule, P; Héno, P; Morand, J J; Mafart, B; La Folie, T; Varlet, P; Mioulet, D; Fourcade, L

    2006-10-01

    Systemic cholesterol embolism is a rare complication of atherosclerosis, and has various presentations. Arterial catheterisms are a common cause. However, the association with an aortic dissection has been exceptionally reported. We report the observation of a 70 year-old man, with coronary artery disease, hypertension, diabetes and dyslipidemia. Six months before hospitalization, a coronary angioplasty was performed due to recurrent angina. The association of purpuric lesions on the feet, with acute renal failure confirmed cholesterol embolism syndrome. Transoesophageal echocardiography showed a dissection of the descending thoracic aorta associated with complex atheroma. The evolution was marked by the pulpar necrosis of a toe and by a worsening of the renal failure, requiring definitive hemodialysis. Further echographic control highlighted the rupture of the intimal veil of the dissection. Cholesterol embolism syndrome may reveal an aortic dissection in patients without thoracic symptoms. In such cases, transoesophageal echocardiography is a useful and non-invasive examination.

  16. In-plane elastic stability of fixed parabolic shallow arches

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    The nonlinear behavior of fixed parabolic shallow arches subjected to a vertical uniform load is inves- tigated to evaluate the in-plane buckling load. The virtual work principle method is used to establish the non-linear equilibrium and buckling equations. Analytical solutions for the non-linear in-plane sym- metric snap-through and antisymmetric bifurcation buckling loads are obtained. Based on the least square method, an approximation for the symmetric buckling load of fixed parabolic arch is proposed to simplify the solution process. And the relation between modified slenderness and buckling modes are discussed. Comparisons with the results of finite element analysis demonstrate that the solutions are accurate. A cable-arch structure is presented to improve the in-plane stability of parabolic arches. The comparison of buckling loads between cable-arch systems and arches only show that the effect of cables becomes more evident with the increase of arch’s modified slenderness.

  17. Accuracy of impressions obtained with dual-arch trays.

    Science.gov (United States)

    Wöstmann, Bernd; Rehmann, Peter; Balkenhol, Markus

    2009-01-01

    This study aimed to analyze the accuracy resulting from dual-arch impressions when compared to conventional impressions in complex preparations (ie, inlay and partial crown). One hundred eighty impressions were made using two different dual-arch trays; conventional trays served as the control. The accuracy of the dies obtained (Fuji-Rock EP, GC Europe) was assessed indirectly from the change of 59 transversal dimensions. Statistical analysis (t test, analysis of variance) revealed that less rigid dual-arch trays performed better than rigid ones. Though the inlay preparation was more difficult to reproduce with dual-arch trays, it can be concluded that the accuracy obtainable with nonrigid dual-arch trays is comparable to impressions taken from full-arch trays.

  18. Asymptotics of the QMLE for General ARCH(q) Models

    DEFF Research Database (Denmark)

    Kristensen, Dennis; Rahbek, Anders Christian

    2009-01-01

    Asymptotics of the QMLE for Non-Linear ARCH Models Dennis Kristensen, Columbia University Anders Rahbek, University of Copenhagen Abstract Asymptotic properties of the quasi-maximum likelihood estimator (QMLE) for non-linear ARCH(q) models -- including for example Asymmetric Power ARCH and log......-ARCH -- are derived. Strong consistency is established under the assumptions that the ARCH process is geometrically ergodic, the conditional variance function has a finite log-moment, and finite second moment of the rescaled error. Asymptotic normality of the estimator is established under the additional assumption...... that certain ratios involving the conditional variance function are suitably bounded, and that the rescaled errors have little more than fourth moment. We verify our general conditions, including identification, for a wide range of leading specific ARCH models....

  19. Anatomic variations of the branches of the aortic arch in a Peruvian population

    Directory of Open Access Journals (Sweden)

    Julio Arturo Huapaya

    2015-07-01

    Full Text Available INTRODUCCIÓN Reportes previos en dos países de América del Sur encontraron una variante anatómica que no había sido reportada en el resto del mundo, lo que podría dar indicios acerca de una diferencia racial. OBJETIVO Describir las variaciones en la distribución anatómica de las ramas del arco aórtico en una población peruana. MÉTODOS Estudio retrospectivo de una serie de casos de personas en quienes se realizó una angiografía por tomografía de la aorta torácica. Se analizaron los informes que registraron la descripción de las variaciones de las ramas del arco aórtico, basados en los ocho tipos descritos por la literatura científica. RESULTADOS Se analizaron 361 informes. Se encontró que 282 pacientes (78,12% tuvieron la configuración clásica (tipo I, arco aórtico que da origen al tronco braquiocefálico, a la carótida común izquierda y a la subclavia izquierda, seguido por el tipo II (arteria carótida común izquierda como rama del tronco braquiocefálico con 41 pacientes (11,36%, y el tipo IX (un ostium común para el tronco braquiocefálico y la carótida común izquierda con 25 pacientes (6,93%. Este último y otros dos tipos resultaron ser nuevas variantes del arco aórtico. CONCLUSIÓN En esta serie de casos peruana, los tipos de arco aórtico I, II y IX fueron los más frecuentes. Adicionalmente, se encontraron otros dos tipos nuevos que no habían sido descritos en la literatura previamente. Es necesario profundizar la investigación sobre estas variantes para evaluar el factor racial en Sudamérica y una posible relación con eventos clínicos o quirúrgicos.

  20. Collaterals branches of the aortic arch and its main rami in rabbit (Oryctolagus cuniculus)

    OpenAIRE

    Souza, Fernanda de; Bavaresco, Andréia Zechin; Campos, Rui

    2013-01-01

    Neste trabalho, sistematizaram-se os ramos colaterais do arco aórtico e suas principais ramifi cações, utilizando 30 coelhos da raça Nova Zelândia. O sistema arterial foi preenchido com látex corado em vermelho através da aorta torácica no sentido contrário ao fl uxo sanguíneo e fi xado em uma solução aquosa de formaldeído a 20%. Os ramos colaterais do arco aórtico foram o tronco braquiocefálico e a artéria subclávia esquerda. O tronco braquiocefálico emitiu as artérias carótidas comuns esque...

  1. GenTAC Registry Report: Gender Differences Among Individuals with Genetically-Triggered Thoracic Aortic Aneurysm and Dissection

    Science.gov (United States)

    Holmes, Kathryn W.; Maslen, Cheryl L.; Kindem, Mark; Kroner, Barbara L.; Song, Howard K.; Ravekes, William; Dietz, H.C.; Weinsaft, Jonathan W.; Roman, Mary J.; Devereux, Richard B.; Pyeritz, Reed E.; Bavaria, Joseph; Milewski, Karianna; Milewicz, Dianna; LeMaire, Scott A.; Hendershot, Tabitha; Eagle, Kim A.; Tolunay, H. Eser; Desvigne-Nickens, Patrice; Silberbach, Michael

    2013-01-01

    Previous data suggest women are at increased risk of death from aortic dissection. Therefore, we analyzed data from the GenTAC registry, the NIH-sponsored program that collects information about individuals with genetically-triggered thoracic aortic aneurysms and cardiovascular conditions. We performed cross-sectional analyses in adults with Marfan syndrome (MFS), familial thoracic aortic aneurysm or dissection (FTAAD), bicuspid aortic valve (BAV) with thoracic aortic aneurysm or dissection, and subjects under 50 years of age with thoracic aortic aneurysm or dissection (TAAD<50y). Women comprised 32% of 1449 subjects and were 21% of subjects with BAV, 34% with FTAAD, 22% with TAAD <50y, and 47% with MFS. Thoracic aortic dissections occurred with equal gender frequency yet women with BAV had more extensive dissections. Aortic size was smaller in women but was similar after controlling for BSA. Age at operation for aortic valve dysfunction, aneurysm or dissection did not differ by gender. Multivariate analysis (adjusting for age, BSA, hypertension, study site, diabetes, and subgroup diagnoses) showed that women had fewer total aortic surgeries (OR= 0.65, p < 0.01) and were less likely to receive angiotensin converting enzyme inhibitors (ACEi) (OR=0.68, p < 0.05). As in BAV, other genetically-triggered aortic diseases such as FTAAD and TAAD<50 are more common in males. In women, decreased prevalence of aortic operations and less treatment with ACEi may be due to their smaller absolute aortic diameters. Longitudinal studies are needed to determine if women are at higher risk for adverse events. PMID:23444191

  2. Valve-in-Valve Replacement Using a Sutureless Aortic Valve

    Science.gov (United States)

    Dohmen, Pascal M.; Lehmkuhl, Lukas; Borger, Michael A.; Misfeld, Martin; Mohr, Friedrich W.

    2016-01-01

    Patient: Female, 61 Final Diagnosis: Tissue degeneration Symptoms: Dyspnea Medication: — Clinical Procedure: Redo valve replacement Specialty: Surgery Objective: Rare disease Background: We present a unique case of a 61-year-old female patient with homograft deterioration after redo surgery for prosthetic valve endocarditis with root abscess. Case Report: The first operation was performed for type A dissection with root, arch, and elephant trunk replacement of the thoracic aorta. The present re-redo surgery was performed as valve-in-valve with a sutureless aortic biopros-thesis. The postoperative course was uneventful and the patient was discharged on day 6. Conclusions: The current case report demonstrates that sutureless bioprostheses are an attractive option for surgical valve-in-valve procedures, which can reduce morbidity and mortality. PMID:27694795

  3. Abdominal aortic aneurysm surgery

    DEFF Research Database (Denmark)

    Gefke, K; Schroeder, T V; Thisted, B

    1994-01-01

    The goal of this study was to identify patients who need longer care in the ICU (more than 48 hours) following abdominal aortic aneurysm (AAA) surgery and to evaluate the influence of perioperative complications on short- and long-term survival and quality of life. AAA surgery was performed in 553......, 78% stated that their quality of life had improved or was unchanged after surgery and had resumed working. These data justify a therapeutically aggressive approach, including ICU therapy following AAA surgery, despite failure of one or more organ systems....

  4. Mathematical model of a moment-less arch.

    Science.gov (United States)

    Lewis, W J

    2016-06-01

    This paper presents a mathematical model for predicting the geometrical shapes of rigid, two-pin, moment-less arches of constant cross section. The advancement of this work lies in the inclusion of arch self-weight and the ability to produce moment-less arch forms for any span/rise ratio, and any ratio of uniformly distributed load per unit span, w, to uniformly distributed arch weight per unit arch length, q. The model is used to derive the shapes of two classical 'moment-less' arch forms: parabolic and catenary, prior to demonstrating a general case, not restricted by the unrealistic load assumptions (absence of q, in the case of a parabolic form, or no w, in the case of a catenary arch). Using the same value of span/rise ratio, and w/q>1, the behaviour of the moment-less and parabolic arches under permanent loading, (w+q), is analysed. Results show the former to be developing much lower stresses than its parabolic rival, even when there are relatively small differences in the two geometries; for a medium span/rise ratio of 4 and w/q=2, differences in the parabolic and moment-less arch geometries would, in practical terms, be viewed as insignificant, but the stresses in them are different.

  5. An Electrostatically Actuated MEMS Arch Band-Pass Filter

    Directory of Open Access Journals (Sweden)

    Hassen M. Ouakad

    2013-01-01

    Full Text Available This work presents an investigation of the dynamics of micromachined arches resonators and their potential to be utilized as band-pass filters. The arches are actuated by a DC electrostatic load superimposed to an AC harmonic load. The dynamic response of the arch is studied analytically using a Galerkin-based reduced-order model when excited near its fundamental and third natural frequencies. Several simulation results are presented demonstrating interesting jumps and snap-through behavior of the arches and their attractive features for uses as band-pass filters, such as their sharp roll-off from pass bands to stop bands and their flat response.

  6. Transcatheter aortic valve implantation vs. surgical aortic valve replacement for treatment of severe aortic stenosis

    DEFF Research Database (Denmark)

    Siontis, George C M; Praz, Fabien; Pilgrim, Thomas

    2016-01-01

    AIMS: In view of the currently available evidence from randomized trials, we aimed to compare the collective safety and efficacy of transcatheter aortic valve implantation (TAVI) vs. surgical aortic valve replacement (SAVR) across the spectrum of risk and in important subgroups. METHODS AND RESULTS...

  7. [Aortic valve replacement for the small aortic annulus].

    Science.gov (United States)

    Oshima, H; Usui, A; Akita, T; Ueda, Y

    2006-04-01

    Aortic valve surgery for the small aortic annulus is still challenging for surgeons. Recently, the new types of high performance prosthesis have been developed and the chance of an aortic root enlargement (ARE) is decreasing. In this study, we propose the ideal strategy of the aortic surgery for the small aortic annulus. We analyzed the clinical records of 158 patients who underwent aortic valve replacement from August 1999 to October 2005 in our institution. The small aortic annulus was observed in 38 patients (24%). Fourteen patients of this group underwent ARE. Patient-prosthesis mismatch (PPM) was less frequently observed in patients with ARE compared to those without ARE. The additional time required for ARE was not considerable, and neither ischemic time nor cardiopulmonary bypass time was significantly prolonged by ARE. In conclusion, we have to select a prosthesis with sufficient orifice area to avoid PPM, otherwise we should choose an option of ARE. For this consideration, we definitely need the chart that demonstrates the relationship between the nominal size of various types of prostheses and the size of a patient's annulus that those prostheses actually fit.

  8. Valve-sparing aortic root replacement and aortic valve repair in a patient with acromegaly and aortic root dilatation

    Directory of Open Access Journals (Sweden)

    Karel Van Praet

    2015-07-01

    Full Text Available Aortic regurgitation and dilatation of the aortic root and ascending aorta are severe complications of acromegaly. The current trend for management of an aortic root aneurysm is valve-sparing root replacement as well as restoring the diameter of the aortic sinotubular junction (STJ and annulus. Our case report supports the recommendation that in patients with acromegaly, severe aortic root involvement may indicate the need for surgery.

  9. A Functional Version of the ARCH Model

    CERN Document Server

    Hormann, Siegfried; Reeder, Ron

    2011-01-01

    Improvements in data acquisition and processing techniques have lead to an almost continuous flow of information for financial data. High resolution tick data are available and can be quite conveniently described by a continuous time process. It is therefore natural to ask for possible extensions of financial time series models to a functional setup. In this paper we propose a functional version of the popular ARCH model. We will establish conditions for the existence of a strictly stationary solution, derive weak dependence and moment conditions, show consistency of the estimators and perform a small empirical study demonstrating how our model matches with real data.

  10. A Binomial Integer-Valued ARCH Model.

    Science.gov (United States)

    Ristić, Miroslav M; Weiß, Christian H; Janjić, Ana D

    2016-11-01

    We present an integer-valued ARCH model which can be used for modeling time series of counts with under-, equi-, or overdispersion. The introduced model has a conditional binomial distribution, and it is shown to be strictly stationary and ergodic. The unknown parameters are estimated by three methods: conditional maximum likelihood, conditional least squares and maximum likelihood type penalty function estimation. The asymptotic distributions of the estimators are derived. A real application of the novel model to epidemic surveillance is briefly discussed. Finally, a generalization of the introduced model is considered by introducing an integer-valued GARCH model.

  11. Aortic biomechanics in hypertrophic cardiomyopathy

    Science.gov (United States)

    Badran, Hala Mahfouz; Soltan, Ghada; Faheem, Nagla; Elnoamany, Mohamed Fahmy; Tawfik, Mohamed; Yacoub, Magdi

    2015-01-01

    Background: Ventricular-vascular coupling is an important phenomenon in many cardiovascular diseases. The association between aortic mechanical dysfunction and left ventricular (LV) dysfunction is well characterized in many disease entities, but no data are available on how these changes are related in hypertrophic cardiomyopathy (HCM). Aim of the work: This study examined whether HCM alone is associated with an impaired aortic mechanical function in patients without cardiovascular risk factors and the relation of these changes, if any, to LV deformation and cardiac phenotype. Methods: 141 patients with HCM were recruited and compared to 66 age- and sex-matched healthy subjects as control group. Pulse pressure, aortic strain, stiffness and distensibility were calculated from the aortic diameters measured by M-mode echocardiography and blood pressure obtained by sphygmomanometer. Aortic wall systolic and diastolic velocities were measured using pulsed wave Doppler tissue imaging (DTI). Cardiac assessment included geometric parameters and myocardial deformation (strain and strain rate) and mechanical dyssynchrony. Results: The pulsatile change in the aortic diameter, distensibility and aortic wall systolic velocity (AWS') were significantly decreased and aortic stiffness index was increased in HCM compared to control (P < .001) In HCM AWS' was inversely correlated to age(r = − .32, P < .0001), MWT (r = − .22, P < .008), LVMI (r = − .20, P < .02), E/Ea (r = − .16, P < .03) LVOT gradient (r = − 19, P < .02) and severity of mitral regurg (r = − .18, P < .03) but not to the concealed LV deformation abnormalities or mechanical dyssynchrony. On multivariate analysis, the key determinant of aortic stiffness was LV mass index and LVOT obstruction while the role LV dysfunction in aortic stiffness is not evident in this population. Conclusion: HCM is associated with abnormal aortic mechanical properties. The severity of cardiac

  12. Goldenhar Syndrome in Association with Duane Syndrome

    Directory of Open Access Journals (Sweden)

    U D Shrestha

    2012-03-01

    Full Text Available Goldenhar syndrome (GHS is also known as Oculo-Auriculo-Vertebral (OAV syndrome or Branchial arch syndrome. Duane retraction syndrome (DRS is a congenital disorder of ocular motility characterized by limited abduction, adduction or both. It is unilateral in 80% of cases. The important and interesting part of this eight months old child is presence of GHS with DRS. She has bilateral invol-vement, which is seen in only 5-8% of GHS, as compared to high incidence of unilateral involve-ment. This child also had refractive error of + 6.00/ - 1.5 * 180. At four year of age her vision with glass was 6/9. Children with GHS and DRS should have early eye examination done to treat the problem of refractive error. Keywords: Duane retraction syndrome; goldenhar syndrome, refractive error.

  13. 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.

  14. Three-dimensional measurement of foot arch in preschool children

    Directory of Open Access Journals (Sweden)

    Chang Hsun-Wen

    2012-09-01

    Full Text Available Abstract Background The prevalence of flexible flatfoot is high among preschool-aged children, but the effects of treatment are inconclusive due to the unclear definitions of normal flatfoot. To date, a universally accepted evaluation method of the foot arch in children has not been completely established. Our aims of this study were to establish a new method to evaluate the foot arch from a three dimensional perspective and to investigate the flexibility of the foot arch among children aged from two to six. Methods A total of 44 children aged from two to six years of age were put into five age groups in this study. The navicular height was measured with one leg standing, and both feet were scanned separately in both sitting and one leg standing positions to compute the foot arch volume. The arch volume index, which represents the ratio of the difference in volume between sitting and one leg standing positions to the volume when sitting was calculated to demonstrate the flexibility of the foot arch. The differences of measured parameters between each aged group were analyzed by one-way ANOVA. Results The arch volumes when sitting and standing were highly correlated with the navicular height. The navicular height ranged from 15.75 to 27 mm, the arch volume when sitting ranged from 6,223 to 11,630 mm3, and the arch volume when standing from 3,111 to 7,848 mm3 from two to six years of age. The arch volume index showed a declining trend as age increased. Conclusion This study is the first to describe the foot arch with volume perspective in preschool-aged children. The foot arch volume was highly correlated with the navicular height. Research results show both navicular height index and arch volume index gradually increase with age from two to six. At the same time the arch also becomes rigid with age from two to six. These results could be applied for clinical evaluation of the foot arch and post-treatment evaluation.

  15. Self-gated CINE MRI for combined contrast-enhanced imaging and wall-stiffness measurements of murine aortic atherosclerotic lesions.

    Directory of Open Access Journals (Sweden)

    Brigit den Adel

    Full Text Available BACKGROUND: High-resolution contrast-enhanced imaging of the murine atherosclerotic vessel wall is difficult due to unpredictable flow artifacts, motion of the thin artery wall and problems with flow suppression in the presence of a circulating contrast agent. METHODS AND RESULTS: We applied a 2D-FLASH retrospective-gated CINE MRI method at 9.4T to characterize atherosclerotic plaques and vessel wall distensibility in the aortic arch of aged ApoE(-/- mice after injection of a contrast agent. The method enabled detection of contrast enhancement in atherosclerotic plaques in the aortic arch after I.V. injection of micelles and iron oxides resulting in reproducible plaque enhancement. Both contrast agents were taken up in the plaque, which was confirmed by histology. Additionally, the retrospective-gated CINE method provided images of the aortic wall throughout the cardiac cycle, from which the vessel wall distensibility could be calculated. Reduction in plaque size by statin treatment resulted in lower contrast enhancement and reduced wall stiffness. CONCLUSIONS: The retrospective-gated CINE MRI provides a robust and simple way to detect and quantify contrast enhancement in atherosclerotic plaques in the aortic wall of ApoE(-/- mice. From the same scan, plaque-related changes in stiffness of the aortic wall can be determined. In this mouse model, a correlation between vessel wall stiffness and atherosclerotic lesions was found.

  16. The Medium-Term Effects of Treatment for Mild Aortic Recoarctation

    DEFF Research Database (Denmark)

    Grøndahl, Camilla; Pedersen, Thais Almeida Lins; Hjortdal, Vibeke Elisabeth

    2017-01-01

    OBJECTIVE: To assess medium-term effects of treatment for mild to moderate recoarctation in an adult population. DESIGN: We identified all patients who had coarctation repair between 1965 and 1985 in our institution. They were all (n = 156) invited to a thorough outpatient examination, which...... > .05), and maximum flow velocity in the distal aortic arch decreased (2.8 ± 0.5 m/s vs 2.1 ± 0.4 m/s, P coarctation gradient was significantly reduced (15 ± 7 mm Hg vs 5 ± 9 mm Hg, P coarctation site was increased (11 ± 3 mm vs 15 ± 3 mm, P

  17. Defect in Posterior Arch of Atlas in Myelomeningocele

    NARCIS (Netherlands)

    G. Blaauw (Gerhard)

    1971-01-01

    textabstractThe posterior arches of the cervical vertebrae of 30 children who died with a myelomeningocele in the lower thoracic, lumbar or sacral region were examined; in 70 per cent of these cases a defect was found in the posterior arch of the atlas, which was bridged by a firm fibrous band. Alté

  18. Level-ARCH Short Rate Models with Regime Switching

    DEFF Research Database (Denmark)

    Christiansen, Charlotte

    This paper introduces regime switching volatility into level- ARCH models for the short rates of the US, the UK, and Germany. Once regime switching and level effects are included there are no gains from including ARCH effects. It is of secondary importance exactly how the regime switching is spec...

  19. Twin - Arch technique. Revival of the "edgewise -Technique"?

    Directory of Open Access Journals (Sweden)

    Jacob Karp

    2012-01-01

    The SNB – Bracket brings a new dimension into the orthodontic world which is most apparent in extraction cases. Its Teflon – like material has a very low friction coefficient thus, reducing the treatment time considerably. Through the use of low dimensioned arch wires, the Twin – Arch Technique becomes a Light – wire system and simultaneously provides good anchorage and torque control.

  20. Modelling subset multivariate ARCH model via the AIC principle

    Institute of Scientific and Technical Information of China (English)

    2002-01-01

    In this paper we consider the problem of identifying a parsimonious subset multivariate ARCH model based on the AIC principle. The proposed approach can reduce the number of parameters in the final ARCH specification and allows for non-constant correlations between the components. Some simulation results illustrate the viability of the proposed procedure.

  1. Full-scale load tests of Pearl-Chain arches

    DEFF Research Database (Denmark)

    Halding, Philip Skov; Hertz, Kristian Dahl; Schmidt, Jacob Wittrup

    2017-01-01

    , pre-fabricated, composite, lightweight, concrete deck elements (SL-Decks), that are post-tensioned together into an arch shape. The two Pearl-Chain arches, each with a span of 13m and a rise of 1m, were placed on a post tensioned bearing plate prepared in advance. The aches are tested with load...

  2. Thoracic aortic aneurysm: A rare cause of elevated hemidiaphragm

    Directory of Open Access Journals (Sweden)

    Md Arshad Ejazi

    2016-01-01

    Full Text Available Phrenic nerve palsy causing hemidiaphragm paralysis is a very uncommon feature of thoracic aortic aneurysm. In one case, a 30 year male complained of chronic dull aching chest pain, and hoarseness of voice; posteroanterior view chest radiograph revealed large spherical radiopacity on the left upper lung zone with smooth lobulated margin with elevated left hemidiaphragm. On Colour Doppler sonography, lesion was anechoic on gray scale sonography but on Doppler analysis revealed intense internal vascularity within it with characteristic "Ying Yang" sign. The finding favor the vascular origin of the lesion and a diagnosis of an arterial aneurysm was made Contrast-enhanced computed tomography (CT of the thorax revealed a large well defined spherical lesion of 8 × 10 cm size with smooth well defined margin arising from the aortic arch and attenuation of impending rupture or dissection were lesion on immediate post contrast and delayed scan was similar to that of aorta. Left hemidiaphragm elevation was explained by the gross mass effect of the aneurysm causing right phrenic nerve palsy.

  3. Limitation of imaging in identifying iatrogenic aortic coarctation following thoracic endovascular aortic repair.

    Science.gov (United States)

    Thakkar, Rajiv N; Thomaier, Lauren; Qazi, Umair; Verde, Franco; Malas, Mahmoud B

    2015-04-01

    A 21-year-old male suffered blunt trauma from a motor vehicle accident causing thoracic aorta tear. The smallest available stent graft was deployed. Definitive repair was later performed using a 22 × 22 × 116 mm Talent Thoracic Stent Graft. The postoperative course was uneventful. Seventeen months later, he presented with dizziness, chest pain, acute renal failure, malignant hypertension, and troponin elevation. Computed tomography (CT) angiogram and transesophageal echocardiogram did not reveal any dissection, stent stenosis or collapse. Cardiac catheterization showed normal coronary arteries but a 117 mm Hg gradient across the stent graft. Iatrogenic coarctation of the aorta was confirmed with a second measurement during arch angiogram. A Palmaz stent was deployed over the distal end of the previous stent graft with complete resolution of symptoms and gradual normalization of kidney function. This case report demonstrates a need for wider availability and selecting appropriate stent graft in treating traumatic aortic injuries in young patients. It is the first case report of the inability of current imaging modalities in confirming stent collapse. Pressure gradient is a useful tool in confirming stent collapse when clinical scenario does not match CT findings.

  4. Seismic multi-arch structures in East China

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    In previous papers (Wencai, 2002, 2003), the author has analyzed the deep seismic reflection profiles along some of the Mesozoic plutons in East China, and has pointed out that the crustal structures around these intrusions usually correlate with a special seismic fabric called the seismic multi-arch structure. This paper will continue to show main characteristics of the seismic multi-arch structures and discuss their relationship with mantle-origin magmatism and the lithospheric thinning process. Calibration of seismic reflectors in Chinese continental drilling sites shows that small-scale arch-like reflectors can be generated by fractured eclogites or other plutons, they do not belong to the multi-arch structure specially discussed in the paper. The multi-arch structure is characterized by several arch-like reflectors distributed in both the upper and lower crust with granitoid plutons or stocks exposed on the surface, which do not have obvious negative Europium anomalies. Based on the distribution pattern of arch reflectors, the multi-arch magmatic structures can be divided into three main types, namely the simple vertical combination type, the spread arch magmatic structure and the arch-bouquet structure. All of them correlate to mantle-origin magmatism, but occur in different places. The spread arch magmatic structures occur within a Mesozoic/Cenozoic rift zone with very thin and hot lithosphere. The vertical combination type of the multi-arch structures occurred near the rift zones where lithosphere was thin and hot. The arch-bouquet magmatic structures occur far from the rift zones where the lithosphere is not hot. The continental rifting acted as the late episode of the lithospheric thinning process seeing that the rift zones usually coincide with the thinnest parts of the lithosphere in East China. In different locations within the lithospheric thinning areas, mantle-origin magmatic activities have different characteristics, which might generate different

  5. Atlantoaxial stabilization using multiaxial C-1 posterior arch screws.

    Science.gov (United States)

    Donnellan, Michael B; Sergides, Ioannis G; Sears, William R

    2008-12-01

    The authors present a novel technique of atlantoaxial fixation using multiaxial C-1 posterior arch screws. The technique involves the insertion of bilateral multiaxial C-1 posterior arch screws, which are connected by crosslinked rods to bilateral multiaxial C-2 pars screws. The clinical results are presented in 3 patients in whom anomalies of the vertebral arteries, C-1 lateral masses, and/or posterior arch of C-1 presented difficulty using existing fixation techniques with transarticular screws, C-1 lateral mass screws, or posterior wiring. The C-1 posterior arch screws achieved solid fixation and their insertion appeared to be technically less demanding than that of transarticular or C-1 lateral mass screws. This technique may reduce the risk of complications compared with existing techniques, especially in patients with anatomical variants of the vertebral artery, C-1 lateral masses, or C-1 posterior arch. This technique may prove to be an attractive fixation option in patients with normal anatomy.

  6. Left testicular artery arching over the ipsilateral renal vein

    Institute of Scientific and Technical Information of China (English)

    Munekazu Naito; Hayato Terayama; Yoichi Nakamura; Shogo Hayashi; Takayoshi Miyaki; Masahiro Itoh

    2006-01-01

    Aim: To report two cases of the left testicular artery arching over the left renal vein (LRV) before running downward to the testis. Methods: The subjects were obtained from two Japanese cadavers. During the student course of gross-anatomical dissection, the anatomical relationship between the testicular vessels and the renal vein was specifically observed. Results: The arching left testicular artery arose from the aorta below the LRV and made a loop around the LRV, which appeared to be mildly compressed between the arching artery and the psoas major muscle.Conclusion: Clinically, compression of the LRV between the abdominal aorta and the superior mesenteric artery occasionally induces LRV hypertension, resulting in varicocele, orthostatic protenuria and hematuria. Considering that the incidence of a left arching testicular artery is higher than that of a right one, an arching left artery could be an additional cause of LRV hypertension.

  7. Effect of Obesity on Arch Index in Young Adults

    Directory of Open Access Journals (Sweden)

    Sneha Sameer Ganu

    2013-01-01

    Full Text Available Background: Excessive increases in weight bearing forces caused by obesity may negatively affect the lower limbs and feet but minimal research has examined the long-term loading effects of obesity on the musculoskeletal system, particularly in reference to the feet. Objectives: The purpose of the study was to investigate the effect of obesity on medial longitudinal arch of foot in young adults. Method: 60 subjects, 30 obese & 30 non obese were assessed for height & weight using standard technique. Radiographic images under static condition were used for calculating the arch index. Result: The arch index of obese subjects was significantly lower than the non obese subjects & there is a negative correlation between the BMI & the arch index. Conclusion: These results suggests that obesity lowers the medial longitudinal arch of foot.

  8. Abdominal aortic aneurysms

    DEFF Research Database (Denmark)

    Lindholt, Jes Sanddal

    2010-01-01

    Although the number of elective operations for abdominal aortic aneurysms (AAA) is increasing, the sex- and age-standardised mortality rate of AAAs continues to rise, especially among men aged 65 years or more. The lethality of ruptured AAA continues to be 80-95%, compared with 5-7% by elective....... The acceptance rate was 77%, and 95% accept control scans. Furthermore, persons at the highest risk of having an AAA attend screening more frequently. We found that 97% of the interval cases developed from aortas that initially measured 2.5-2.9 cm - i.e. approx. only 5% attenders need re-screening at 5-year...... methods for measuring the degree of wall calcification must be developed and validated....

  9. CONGENITAL QUADRICUSPID AORTIC-VALVE

    NARCIS (Netherlands)

    BROUWER, MHJ; DEGRAAF, JJ; EBELS, T

    1993-01-01

    Two patients with a quadricuspid aortic valve are described, one of them with concomitant juxtaposed coronary orifices facing the right hand facing sinus. The etiology and incidence of this congenital anomaly will be discussed.

  10. Transcatheter Aortic Heart Valve Thrombosis

    DEFF Research Database (Denmark)

    Hansson, Nicolaj C; Grove, Erik L; Andersen, Henning R;

    2016-01-01

    BACKGROUND: There is increasing focus on transcatheter heart valve (THV) thrombosis. However, there are limited data on incidence, clinical implications and predisposing factors of THV thrombosis following transcatheter aortic valve replacement (TAVR). OBJECTIVES: We assessed the incidence...

  11. Adult thoracic and abdominal aortic

    Directory of Open Access Journals (Sweden)

    Randa O. Kaddah

    2016-06-01

    Conclusion: Aortic COA could be found in any segment of the aorta. Proper identification of the anatomical details and pressure gradient studies are important factors affecting the plan of management.

  12. Minimally invasive aortic valve replacement

    DEFF Research Database (Denmark)

    Foghsgaard, Signe; Schmidt, Thomas Andersen; Kjaergard, Henrik K

    2009-01-01

    In this descriptive prospective study, we evaluate the outcomes of surgery in 98 patients who were scheduled to undergo minimally invasive aortic valve replacement. These patients were compared with a group of 50 patients who underwent scheduled aortic valve replacement through a full sternotomy....... The 30-day mortality rate for the 98 patients was zero, although 14 of the 98 mini-sternotomies had to be converted to complete sternotomies intraoperatively due to technical problems. Such conversion doubled the operative time over that of the planned full sternotomies. In the group of patients whose...... is an excellent operation in selected patients, but its true advantages over conventional aortic valve replacement (other than a smaller scar) await evaluation by means of randomized clinical trial. The "extended mini-aortic valve replacement" operation, on the other hand, is a risky procedure that should...

  13. Professional parachuting: the risk of acute aortic dissection.

    Science.gov (United States)

    Buchholz, Stefan; Quaden, René Bombien; Schmitz, Christoph; Überfuhr, Peter

    2011-09-01

    Acute aortic dissection is a rare disease, but if it occurs rapid diagnosis and therapy are needed. It is usually seen in elderly patients with long-term persistent arterial hypertension. In younger patients, it is mainly caused by congenital connective tissue disorders, such as Marfan syndrome, or by trauma. We present here a 34-year-old male patient with an acute type A aortic dissection. This patient was a professional parachutist and had carried out a large number of parachute jumps during his lifetime. He was admitted to the emergency department with acute chest pain. The symptoms were not related in time to a parachute jump. During a computed tomography scan, an aortic dissection was diagnosed. The patient was immediately referred to the operating room, and the ascending aorta was replaced by a conduit. After a regular postoperative course, the patient was discharged and recovered completely. Although acute aortic dissection is rare in young patients, it has to be considered in cases of acute chest pain. An immediate diagnosis and adequate therapy are essential to offer the patient a good clinical outcome and long-term survival.

  14. Mycotic Saccular Abdominal Aortic Aneurysm in an Infant after Cardiac Catheterization: A Case Report.

    Science.gov (United States)

    Benrashid, Ehsan; McCoy, Christopher C; Rice, Henry E; Shortell, Cynthia K; Cox, Mitchell W

    2015-10-01

    Abdominal aortic aneurysms (AAAs) are a rare entity in the pediatric population. Children with mycotic (infectious) AAA in particular are at risk of life-threatening rupture due to their rapid expansion coupled with aortic wall thinning and deterioration. Here, we present the case of a 10-month-old infant with prior 2-staged repair for hypoplastic left heart syndrome that was incidentally discovered to have a mycotic AAA on abdominal ultrasound (US) for evaluation of renovascular hypertension. Before the time of evaluation with US, the infant had developed methicillin-resistant Staphylococcus aureus bacteremia 3 days after cardiac catheterization with percutaneous thoracic aortic balloon angioplasty. She had normal aortic contours on contrasted computed tomography scan of the abdomen approximately 2 weeks before the aforementioned US evaluation. This infant subsequently underwent open aneurysmorrhaphy with cryopreserved vein patch angioplasty with resolution of her aneurysmal segment.

  15. Thoracic and abdominal aortic diameters in a general population: MRI-based reference values and association with age and cardiovascular risk factors

    Energy Technology Data Exchange (ETDEWEB)

    Mensel, Birger; Hesselbarth, Lydia; Wenzel, Michael; Kuehn, Jens-Peter; Hegenscheid, Katrin [University Medicine Greifswald, Institute of Diagnostic Radiology and Neuroradiology, Greifswald (Germany); Doerr, Marcus [University Medicine Greifswald, Department of Internal Medicine, Greifswald (Germany); DZHK (German Center for Cardiovascular Research), partner site Greifswald, Greifswald (Germany); Voelzke, Henry [University Medicine Greifswald, Institute for Community Medicine, Greifswald (Germany); DZHK (German Center for Cardiovascular Research), partner site Greifswald, Greifswald (Germany); Lieb, Wolfgang [Christian Albrechts University, Institute of Epidemiology, Kiel (Germany); Lorbeer, Roberto [Ludwig-Maximilians-University Hospital, Institute of Clinical Radiology, Munich (Germany)

    2016-04-15

    To generate reference values for thoracic and abdominal aortic diameters determined by magnetic resonance imaging (MRI) and analyse their association with cardiovascular risk factors in the general population. Data from participants (n = 1759) of the Study of Health in Pomerania were used for analysis in this study. MRI measurement of thoracic and abdominal aortic diameters was performed. Parameters for calculation of reference values according to age and sex analysis were provided. Multivariable linear regression models were used for determination of aortic diameter-related risk factors, including smoking, blood pressure (BP), high-density lipoprotein cholesterol (HDL-C). For the ascending aorta (β = -0.049, p < 0.001), the aortic arch (β = -0.061, p < 0.001) and the subphrenic aorta (β = -0.018, p = 0.004), the body surface area (BSA)-adjusted diameters were lower in men. Multivariable-adjusted models revealed significant increases in BSA-adjusted diameters with age for all six aortic segments (p < 0.001). Consistent results for all segments were observed for the positive associations of diastolic BP (β = 0.001; 0.004) and HDL (β = 0.035; 0.087) with BSA-adjusted aortic diameters and for an inverse association of systolic BP (β = -0.001). Some BSA-adjusted median aortic diameters are smaller in men than in women. All diameters increase with age, diastolic blood pressure and HDL-C and decrease as systolic BP increases. (orig.)

  16. Aortic pressure-area relation in Marfan patients with and without β blocking agents: a new non-invasive approach

    NARCIS (Netherlands)

    Nollen, G.J.; Westerhof, B.E.; Groenink, M.; Osnabrugge, A.; Wall, E.E. van der; Mulder, B.J.M.

    2004-01-01

    Objective: To investigate the heterogeneous response to β blockade in patients with Marfan syndrome by non-invasive assessment of the aortic pressure-area curve. Design and patients: 25 patients with the Marfan syndrome who used β blocking agents (mean (SD) age, 29 (10) years; 20 men, five women), s

  17. Loose anagen hair syndrome associated with colobomas and dysmorphic features

    DEFF Research Database (Denmark)

    Hansen, Lars Kjaersgård; Brandrup, Flemming; Clemmensen, Ole

    2004-01-01

    Loose anagen hair syndrome is an uncommon congenital disorder. It may occur in association with other syndromes and dysmorphic features. We report a girl who fulfilled the diagnostic criteria for this syndrome as proposed by Tosti (Arch Dermatol 2002, 138: 521-522). She also had several other...

  18. Evolution of surgical therapy for Stanford acute type A aortic dissection.

    Science.gov (United States)

    Chiu, Peter; Miller, D Craig

    2016-07-01

    Acute type A aortic dissection (AcA-AoD) is a surgical emergency associated with very high morbidity and mortality. Unfortunately, the early outcome of emergency surgical repair has not improved substantially over the last 20 years. Many of the same debates occur repeatedly regarding operative extent and optimal conduct of the operation. The question remains: are patients suffering from too large an operation or too small? The pendulum favoring routine aortic valve resuspension, when feasible, has swung towards frequent aortic root replacement. This already aggressive approach is now being challenged with the even more extensive valve-sparing aortic root replacement (V-SARR) in selected patients. Distally, open replacement of most of the transverse arch is best in most patients. The need for late aortic re-intervention has not been shown to be affected by more extensive distal operative procedures, but the contemporary enthusiasm for a distal frozen elephant trunk (FET) only seems to build. It must be remembered that the first and foremost goal of the operation is to have an operative survivor; additional measures to reduce late morbidity are secondary aspirations. With increasing experience, true contraindications to emergency surgical operation have dwindled, but patients with advanced age, multiple comorbidities, and major neurological deficits do not fare well. The endovascular revolution, moreover, has spawned innovative options for modern practice, including ascending stent graft and adaptations of the old flap fenestration technique. Despite the increasingly complex operations and ever expanding therapies, this life-threatening disease remains a stubborn challenge for all cardiovascular surgeons. Development of specialized thoracic aortic teams and regionalization of care for patients with AcA-AoD offers the most promise to improve overall results.

  19. Twins with progressive thoracic aortic aneurysm, recurrent dissection and ACTA2 mutation.

    Science.gov (United States)

    Ware, Stephanie M; Shikany, Amy; Landis, Benjamin J; James, Jeanne F; Hinton, Robert B

    2014-10-01

    Thoracic aortic aneurysm (TAA) is a genetically mediated disease with variable age of onset. In the pediatric age range, nonsyndromic TAA frequently has a milder course than syndromic forms of TAA, such as Marfan syndrome or Loeys-Dietz syndrome. Herein, we describe 17-year-old identical twin brothers with severe progressive TAA due to a novel de novo ACTA2 mutation. Interestingly, both boys were diagnosed at age 11 with congenital mydriasis, a recently recognized manifestation of some ACTA2 mutations due to smooth muscle dysfunction. One of the brothers presented with acute-onset lower back pain that was identified as dissection of an abdominal aortic aneurysm. Imaging of the chest at this time showed severe fusiform TAA. Cardiac imaging in his twin showed similar TAA, but no abdominal aortic aneurysm. Both brothers underwent valve-sparing aortic root replacement, but have had progressive aortic disease with recurrent dissection requiring multiple surgeries. This case emphasizes the importance of identifying physical stigmata of smooth muscle dysfunction, such as mydriasis, as potential markers for associated aortopathy and vascular diseases.

  20. Dexamethasone normalizes aberrant elastic fiber production and collagen 1 secretion by Loeys-Dietz syndrome fibroblasts: a possible treatment?

    Science.gov (United States)

    Barnett, Christopher P; Chitayat, David; Bradley, Timothy J; Wang, Yanting; Hinek, Aleksander

    2011-06-01

    Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder characterized by facial dysmorphism, cleft palate, dilation of the aortic arch, blood vessel tortuosity and a high risk of aortic dissection. It is caused by mutations in the transforming growth factor β-receptor 1 and 2 (TGFβ-R1 and TGFβ-R2) genes. Fibroblasts derived from 12 Loeys-Dietz syndrome patients, six with TGFB-R1 mutations and six with TGFB-R2 mutations, were analyzed using RT-PCR, biochemical assays, immunohistochemistry and electron microscopy for production of elastin, fibrillin 1, fibulin 1 and fibulin 4 and deposition of collagen type I. All LDS fibroblasts with TGFβ-R1 mutations demonstrated decreased expression of elastin and fibulin 1 genes and impaired deposition of elastic fibers. In contrast, fibroblasts with TGFβ-R2 mutations consistently demonstrated intracellular accumulation of collagen type I in the presence of otherwise normal elastic fiber production. Treatment of the cell cultures with dexamethasone induced remarkable upregulation in the expression of tropoelastin, fibulin 1- and fibulin 4-encoding mRNAs, leading to normalization of elastic fiber production in fibroblasts with TGFβ-R1 mutations. Treatment with dexamethasone also corrected the abnormal secretion of collagen type I from fibroblasts with TGFβ-R2 gene mutations. As the organogenesis-relevant elastic fiber production occurs exclusively in late fetal and early neonatal life, these findings may have implications for treatment in early life. Further studies are required to determine if dexamethasone treatment of fetuses prenatally diagnosed with LDS would prevent or alleviate the connective tissue and vascular defects seen in this syndrome.