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Sample records for aortic arch syndromes

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

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

    2009-05-01

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

  2. 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.

  3. Aortic arch malformations

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    Kellenberger, Christian J.

    2010-01-01

    Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewed. (orig.)

  4. Persistent Fifth Aortic Arch Associated with 22q11.2 Deletion Syndrome

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    Meng-Luen Lee

    2006-01-01

    Conclusion: Congenital conotruncal malformations, including tetralogy of Fallot with pulmonary atresia or stenosis, and aortic arch anomalies including a persistent fifth aortic arch or a right aortic arch, should lead to suspicion of chromosome 22q11.2 deletion when manifested together with any one of the other four cardinal phenotypic features.

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

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

    2009-01-01

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

  6. Double aortic arch

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    Surgery can be done to fix double aortic arch. The surgeon ties off the smaller branch and separates it from the larger branch. Then the surgeon closes the ends of the aorta with stitches. This relieves pressure on the esophagus and windpipe.

  7. Hypoplasia of the aortic arch

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    Ю. С. Синельников

    2015-10-01

    Full Text Available An aggressive surgical approach to early repair of aortic coarctation and concomitant arch hypoplasia results in considerable improvement of the long-term outcomes of surgical treatment by reducing the number of aortic re-coarctation and arterial hypertension persistence events. At present, there exist some methods of determining the degree of aortic arch hypoplasia, which allow for arch reconstruction planning. Our review focuses on the analysis of modern methods of determination of the aortic arch hypoplasia and the outcomes of surgical treatment of this disease.

  8. [A re-operative case of bentall operation and aortic arch replacement using a stent graft for a Marfan syndrome, post sternum turnover and post mitral valve replacement].

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    Sasaki, H; Aomi, S; Noji, S; Uwabe, K; Kihara, S; Kurihara, H; Koyanagi, H

    2001-09-01

    A 36-year-old male with Marfan syndrome succesfully underwent Bentall operation and aortic arch replacement using a stent graft as an elephant trunk. He had received MVR with sternum turn over 14 years previously. Median sternotomy was performed. Under circulatory arrest with rertograde cerebral perfusion we performed Bentall operation and aortic arch replacement using a stent graft. The sternum was cured well. Retractive breathing was not detected. This surgical procedure was effective for cardiovascular disease with Marfan syndrome.

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

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    Karasaki, Takahiro; Tanaka, Makoto

    2015-01-01

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

  10. [Aortic arch advancement surgery as treatment for aortic coarctation with hypoplastic aortic arch in children].

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    Palacios-Macedo-Quenot, Alexis; Urencio, Miguel; Ponce-De-León-Rosales, Sergio; López-Terrazas, Javier; Castañuela-Sánchez, Violeta; March-Mifsut, Almudena; López-Magallón, Alejandro; Pérez-Juárez, Fabiola; Cedillo-Rendón, Irma; Tamariz-Cruz, Orlando

    2012-01-01

    Treatment of aortic coarctation with hypoplastic aortic arch is still a surgical challenge. The aortic arch advancement surgery has shown less re-coarctation frequency. To determine the re-coarctation frequency in patients who underwent aortic arch advancement technique for aortic coarctation with hypoplastic aortic arch and analyze the results. Retrospective and observational study of 38 patients who underwent aortic arch advancement in a third level Institution from 2002 to 2010. Twenty four males and 14 females all with aortic arch Z index diameter of coarctation was O%. With the previously mentioned technique the recoarctation frequency on medium and long term basis was 0%. From the anatomical and functional point of view, we believe this technique offers the best possible results.

  11. Cervical aortic arch and a new type of double aortic arch. Report of a case.

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    Cornali, M; Reginato, E; Azzolina, G

    1976-09-01

    A case of cervical aortic arch is reported. To the best of our knowledge, it is the first to be associated with a serious intracardiac anomaly. In addition, it is part of a new type of double aortic arch, caused by failure of reabsorption of both dorsal aortic roots and persistence of the fourth right and second (or third) left branchial arches.

  12. Ortner’s syndrome: Cardiovocal syndrome caused by aortic arch ps

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    Adil H. Al Kindi

    2016-10-01

    Ortner’s syndrome describes vocal changes caused by cardiovascular pathology. It should be included in the differential diagnosis of patients with cardiovascular risk factors presenting with hoarseness. This case demonstrates the use of endovascular stents to treat the causative pathology with resolution of symptoms. In expert hands, it represents low risk, minimally invasive therapeutic strategy with excellent early results in patients who are high risk for open procedure.

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

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

    2015-07-01

    Middle aortic syndrome (MAS) is a rare clinical entity in childhood, characterized by a severe narrowing of the distal thoracic and/or abdominal aorta, and associated with significant morbidity and mortality. MAS remains a relatively poorly defined disease. This paper systematically reviews the current knowledge on MAS with respect to etiology, clinical impact, and therapeutic options. A systematic search of 3 databases (Embase, MEDLINE, and Cochrane Central Register of Controlled Trials) yielded 1,252 abstracts that were screened based on eligibility criteria resulting in 184 full-text articles with 630 reported cases of childhood MAS. Data extracted included patient characteristics, clinical presentation, vascular phenotype, management, and outcomes. Most cases of MAS are idiopathic (64%), 15% are associated with Mendelian disorders, and 17% are related to inflammatory diseases. Extra-aortic involvement including renal (70%), superior mesenteric (30%), and celiac (22%) arteries is common, especially among those with associated Mendelian disorders. Inferior mesenteric artery involvement is almost never reported. The majority of cases (72%) undergo endovascular or surgical management with residual hypertension reported in 34% of cases, requiring medication or reintervention. Clinical manifestations and extent of extra-aortic involvement are lacking. MAS presents with significant involvement of visceral arteries with over two thirds of cases having renal artery stenosis, and one third with superior mesenteric artery stenosis. The extent of disease is worse among those with genetic and inflammatory conditions. Further studies are needed to better understand etiology, long-term effectiveness of treatment, and to determine the optimal management of this potentially devastating condition. © American Journal of Hypertension, Ltd 2015. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  14. 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.

  15. An Adult Case of Chromosome 22q11.2 Deletion Syndrome Associated with a High-positioned Right Aortic Arch.

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    Hoshino, Yoichi; Machida, Moriya; Shimano, Shun-Ichi; Taya, Teizo

    2017-01-01

    Chromosome 22q11.2 deletion syndrome (22q11.2 DS) has a very wide phenotypic spectrum that includes dysmorphic features, cardiac anomalies, and hypocalcemia arising from hypoparathyroidism. We herein describe an adult case of 22q11.2 DS with associated hypoparathyroidism and anomalies of the aortic arch. Because the patient had been diagnosed with primary hypoparathyroidism at another hospital, a diagnosis of 22q11.2 DS had been overlooked. A chest X-ray examination revealed widening of the mediastinum caused by a high-positioned right aortic arch, and we subsequently confirmed a diagnosis of 22q11.2 DS using fluorescence in situ hybridization. Because primary hypoparathyroidism is a rare disorder, physicians should be aware of the variable phenotypic features of 22q11.2 DS.

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

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

    2017-09-01

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

  17. Prenatal diagnosis of 22q11.2 deletion syndrome associated with right aortic arch, left ductus arteriosus, cardiomegaly, and pericardial effusion.

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    Chen, Yen-Ni; Chen, Chih-Ping; Ko, Tsang-Ming; Wang, Liang-Kai; Wu, Pei-Chen; Chang, Tung-Yao; Wu, Peih-Shan; Yang, Chien-Wen; Wang, Wayseen

    2016-02-01

    To report prenatal diagnosis of 22q11.2 deletion syndrome with right aortic arch (RAA), left ductus arteriosus, cardiomegaly, and pericardial effusion in the fetus. A 35-year-old woman, gravida 2, para 1, was referred to the hospital at 31 weeks of gestation because of abnormal ultrasound findings and whole-genome array comparative genomic hybridization report. G-banding chromosome analysis revealed a karyotype of 46,XX. Level II ultrasound at 22 weeks of gestation revealed RAA with the presence of the aortic arch on the right side of trachea at three vessels and trachea view, left ductus arteriosus, and mild right side pyelectasis. Cardiomegaly and pericardial effusion were also found 2 months later. Array comparative genomic hybridization detected a 2.743-Mb deletion at 22q11.2 region. Multiplex ligation-dependent amplification detected deletion in the DiGeorge syndrome critical region of chromosome 22 low copy number repeat 22-A-C. Metaphase fluorescence in situ hybridization on lymphocyte in cord blood confirmed deletion in 22q11.2 region. Chromosome abnormalities have been found in patients with RAA. Prenatal diagnosis of RAA with or without intracardiac or extracardiac anomalies should include a diagnosis of 22q11.2 deletion syndrome. Copyright © 2016. Published by Elsevier B.V.

  18. In situ total aortic arch replacement for infected distal aortic arch aneurysms with penetrating atherosclerotic ulcer.

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    Okada, Kenji; Yamanaka, Katsuhiro; Sakamoto, Toshihito; Inoue, Takeshi; Matsumori, Masamichi; Kawakami, Fumi; Okita, Yutaka

    2014-11-01

    We present a series of patients who underwent in situ total aortic arch replacement for infected distal aortic arch aneurysms. Between 2002 and 2013, 9 patients with infected distal aortic arch aneurysms underwent total aortic arch replacement using antegrade selective cerebral perfusion. There were 4 male and 5 female patients with a mean age of 72.7±9.0 years. All patients had penetrating atherosclerotic ulcer in the distal aortic arch, which formed saccular aneurysms. Four patients had preoperative hoarseness. Maximum preoperative white blood cell count was 10,211±4375/μL, and mean serum C-reactive protein concentration was 12.7±7.2 mg/dL. Causative microorganisms were identified by blood culture or aortic wall culture and were as follows: Candida albicans, Pseudomonas aeruginosa, Edwardsiella tarda, Streptococcus dysgalactiae, Listeria monocytogenes, Staphylococcus aureus (2 cases), and unknown (2 cases). Radical debridement with in situ total aortic arch replacement was performed in all patients, followed by the omental flap grafting in 7 patients. All surgery was performed on an urgent or emergency basis. Average cardiopulmonary bypass time and lower body circulatory arrest time were 199.7±50.7 minutes and 66.6±13.8 minutes, respectively. There was no in-hospital mortality, but 1 patient died of asphyxia 5 months after hospital discharge. Freedom from recurrence of infection was 100%. Surgical treatment with the combination of radical debridement with in situ total aortic arch replacement using antegrade selective cerebral perfusion and omental flap grafting was a reliable procedure for the treatment of infected distal aortic arch aneurysms. Copyright © 2014. Published by Elsevier Inc.

  19. Arch reconstruction with autologous pulmonary artery patch in interrupted aortic arch.

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    Lee, Won-Young; Park, Jeong-Jun

    2014-04-01

    Various surgical techniques have been developed for the repair of an interrupted aortic arch. However, tension and Gothic arch formation at the anastomotic site have remained major problems for these techniques: Excessive tension causes arch stenosis and left main bronchus compression, and Gothic arch configuration is related to cardiovascular complications. To resolve these problems, we adopted a modified surgical technique of distal aortic arch augmentation using an autologous main pulmonary artery patch. The descending aorta was then anastomosed to the augmented aortic arch in an end-to-side manner. Here, we report two cases of interrupted aortic arch that were repaired using this technique.

  20. 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.)

  1. [Single coronary artery and right aortic arch].

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    Martínez-Quintana, Efrén; Rodríguez-González, Fayna

    2015-01-01

    Coronary anomalies are mostly asymptomatic and diagnosed incidentally during coronary angiography or echocardiography. However, they must be taken into account in the differential diagnosis of angina, dyspnea, syncope, acute myocardial infarction or sudden death in young patients. The case is presented of two rare anomalies, single coronary artery originating from right sinus of Valsalva and right aortic arch, in a 65 year-old patient with atherosclerotic coronary artery disease treated percutaneously. Copyright © 2014 Sociedad Española de Arteriosclerosis. Published by Elsevier España. All rights reserved.

  2. [Right lung cancer with right aortic arch].

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    Kawaguchi, Yasuo; Noriyuki, T; Kuroda, Y; Kuranishi, F; Nakahara, M; Fukuda, T; Ishizaki, Y; Hotta, R; Akimoto, E; Mori, H

    2008-02-01

    An abnormal shadow was detected on chest X-ray mass screening in an asymptomatic 63-year-old man. The further examinations revealed the shadow to be primary lung cancer (Rt. S6. adenocarcinoma, cT2N0M0, c-stage IB) with right aortic arch. We used 3 dimentional-computed tomography (3D-CT) to assess an anatomical feature of vessels in detail. The right lower lobectomy and the dissection of medi astinal lymph nodes was performed. We confirmed no abnormal anatomy of pulmonary artery and vein at surgery, and it was possible to perform right lower lobectomy with the common procedure. Since lymph node was found by intraopetrative pathological examination, since no metastasis from interlobar to subcarinal lymph node was found, we did not perform dissection of upper mediastinal dissection, which was equivalent to ND2a lymph nodes dissection of the left lung cancer in General Rule for Clinical and Pathological Record of Lung Cancer. The patient with right aortic arch is known to have variant anatomy of other intrathoracic vessels occasionally. 3D-CT was quite useful in assessing anatomical feature, and enabled us to perform safe operation.

  3. 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.

  4. Comparison of Total Arch and Partial Arch Transposition During Hybrid Endovascular Repair for Aortic Arch Disease.

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    Kang, W C; Ko, Y-G; Oh, P C; Shin, E K; Park, C-H; Choi, D; Youn, Y N; Lee, D Y

    2016-08-01

    Total arch transposition (TAT) during hybrid endovascular repair for aortic arch disease is believed to allow a better landing zone, but also to be associated with higher peri-operative mortality than partial arch transposition (PAT). Information on this issue is limited. This study was a retrospective analysis. All 53 consecutive patients with aortic arch disease (41 males, mean age 65.0 years) who underwent hybrid endovascular repair with TAT (zone 0, n=20) or PAT (zone 1 or 2, n=33) from 2008 to 2014 were analyzed retrospectively. The peri-operative and late outcomes of these two groups were compared. Baseline characteristics, including EuroSCORE II results, were similar in the two groups. After procedures, peri-operative mortalities and stroke rates were similar in the two groups (5.0% vs. 9.1%, p=1.000, and 10.0% vs. 6.1%, p=.627). Interestingly, all four strokes occurred in patients with a type III aortic arch irrespective of transposition type. Primary success rates (80.0% vs. 69.7%, p=.527) and type I endoleak incidences (20.0% vs. 27.3%, p=.744) were not significantly different. During follow up (mean duration 36.9 months), overall survival (89.7% vs. 87.4% at 1 year and 89.7% vs. 79.3% at 3 years; p=.375) and re-intervention free survival rates (78.6% vs. 92.0% at 1 year; 72.0% vs. 62.2% at 3 years, p=.872) were similar in the two groups. Morbidity and mortality were high within the first year of hybrid endovascular therapy for aortic arch disease, implying that candidates for hybrid procedures need to be selected carefully. Hybrid endovascular repair with TAT was found to have peri-operative mortality, stroke, and long-term survival rates comparable with PAT, so hybrid endovascular repair may be considered, irrespective of type of arch reconstruction, when clinically indicated. Copyright © 2016 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

  5. Prosthetic aortic arch replacement. A new experimental technique.

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    Brodman, R F; Estner, S; Passik, C; Goldman, M

    1990-01-01

    An experimental method for rapid replacement of the transverse aortic arch is presented. Branched intraluminal prostheses were fashioned of varying sizes containing ascending and descending aortic limbs and branches for the brachiocephalic and subclavian vessels. Rigid rings were sewn into the ends to allow rapid fixation of the grafts using extraluminal ligatures. In 31 dogs, arch prostheses were inserted using inflow occlusion at normothermia. Twenty-seven animals successfully underwent aortic arch replacement during periods of circulatory arrest from 2.5 to 6.5 minutes. One animal was electively sacrificed 3 years after arch replacement. Various sized aortic arch prostheses manufactured for clinical use would have the potential advantages of technical simplicity of insertion and reduced cardiopulmonary bypass. Alternatively, varying sized tubular grafts for the brachiocephalic branches could be attached to the currently available tubular intraluminal grafts.

  6. 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. © 2011, Copyright the Authors. Artificial Organs © 2011, International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

  7. Endovascular Stent Grafting for Aortic Arch Aneurysm in Aortoiliac Occlusive Disease following Aortic Arch Debranching and Aortobifemoral Reconstruction

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    Didem Melis Oztas

    2017-01-01

    Full Text Available Treatment of thoracic aortic aneurysms constitutes high mortality and morbidity rates despite improvements in surgery, anesthesia, and technology. Endovascular stent grafting may be an alternative therapy with lower risks when compared with conventional techniques. However, sometimes the branches of the aortic arch may require transport to the proximal segments prior to successful thoracic aortic endovascular stent grafting. Atherosclerosis is accounted among the etiology of both aneurysms and occlusive diseases that can coexist in the same patient. In these situations stent grafting may even be more complicated. In this report, we present the treatment of a 92-year-old patient with aortic arch aneurysm and proximal descending aortic aneurysm. For successful thoracic endovascular stent grafting, the patient needed an alternative route other than the native femoral and iliac arteries for the deployment of the stent graft. In addition, debranching of left carotid and subclavian arteries from the aortic arch was also required for successful exclusion of the thoracic aneurysm.

  8. IDIOPATHIC NEONATAL AORTIC ARCH THROMBOSIS: A CASE REPORT

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    Ignazio Lofù

    2014-04-01

    Full Text Available Background: Neonatal aortic arch thrombosis is a rare but life threatening condition. A correct diagnosis and an appropriate therapeutic approach are fundamental to prevent death or severe injuries. Case presentation: we report a case of spontaneous aortic arch thrombosis in a term newborn, who presented with suggestive signs of aortic coarctation immediately after birth. Despite the initiation of anticoagulant therapy, a massive increase in size of thrombus with evidence of coronary involvement was noted. Thrombolytic treatment was performed with thrombus resolution. Death occurred after a few days due to widespread brain haemorrhage. Conclusion: To date, no definitive guidelines have been published for the treatment of neonatal aortic arch thrombosis, and only anecdotal reports are available. A multidisciplinary approach is fundamental in order to evaluate the risk:benefit ratio of proposed medical and surgical interventions. Further studies are needed to improve consensus evidence based guidelines and ensure appropriate approaches to this condition.

  9. Acute aortic syndromes: current status.

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    Ridge, Carole A; Litmanovich, Diana E

    2015-05-01

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

  10. Imaging findings in the right aortic arch with mirror image branching of arch vessels: An unusual cause of dysphagia

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    Guneet Singh

    2015-01-01

    Full Text Available We report a case of a 72-year-old female with a right aortic arch with mirror-image branching of arch vessels presenting with dysphagia, and characteristic images on barium esophagogram, contrast-enhanced computed tomography scan, and magnetic resonance aortography. Right-sided thoracic aortic arch with mirror-image branching of the brachiocephalic vessels causing dysphagia without associated congenital cardiac anomalies is extremely uncommon. Right-sided aortic arch is a rare congenital abnormality with incidence of 0.05-0.1% in the normal population. Anomalies of great vessels are usually incidental findings, because they are asymptomatic. Right aortic arch infrequently presents with a vascular ring that can cause complete or partial obstruction of the trachea and/or esophagus. The understanding of this arch anomaly is based on Edward′s hypothesis about the double arch system during embryonic developmental.

  11. Case of successful prosthetics of aortic arch in a patient with congenital deformation of aortic arch in severe hypothermia and circulatory arrest

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    Bokeria L.A.

    2014-09-01

    Full Text Available Atresia of the aortic arch is a rare congenital disease, often accompanied by congenital heart defects. Isolated atresia of the aorta is rare and usually has a complex deformation with multiple aneurysms, bending of the aorta and its branches. This anatomy complicates the exposure and makes surgery technically complicated with conventional approaches. The aim of the work is the presentation of a case of successful prosthetics of aortic arch and a 5-year observation of a patient with congenital deformation of the aortic arch in severe hypothermia and circulatory arrest. The article presents the variant of surgical tactics in treatment of patients with this form of atresia of aortic arch.

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

    Science.gov (United States)

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

    2017-02-01

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

  13. Total arch and descending thoracic aortic replacement by left thoracotomy.

    Science.gov (United States)

    Corvera, Joel S; Fehrenbacher, John W

    2012-05-01

    The hybrid treatment of transverse aortic arch pathologies with supraaortic debranching and endovascular repair is associated with significant morbidity and death and lacks long-term follow-up. The traditional two-stage open surgical approach to extensive arch and descending thoracic aneurysms carries a significant interval mortality rate. We report the results of a single-stage technique of total arch and descending thoracic aortic replacement by a left thoracotomy. From January 1995 to February 2011, 426 patients underwent thoracic or thoracoabdominal aneurysm repair, of which a highly selected group of 27 patients underwent total arch replacement with descending thoracic or thoracoabdominal aortic replacement. All procedures were performed with hypothermic circulatory arrest and selective antegrade cerebral perfusion. Two patients required transverse division of the sternum. Two patients had emergency or urgent operations. Five patients had concomitant coronary artery bypass, and 1 had concomitant mitral valve replacement. There were no hospital deaths, no cerebrovascular accidents, and one instance of transient spinal cord ischemia. Three patients had acute renal failure not requiring hemodialysis. Intubation in 5 patients exceeded 48 hours, and 1 patient needed tracheostomy. Two patients required reexploration for postoperative bleeding. Survival at 1, 3, and 5 years was 95%, 78%, and 73%, respectively. Replacement of the total arch and descending thoracic aorta by a left thoracotomy provides excellent short-term and long-term results for the treatment of extensive arch and thoracic aortic pathology, without the need for a second-stage operation. Other cardiac pathologies, such as left-sided coronary disease and mitral valve disease, can be addressed concurrently. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  14. Variants of the left aortic arch branches

    African Journals Online (AJOL)

    Type C (Courtesy of Rutherford. Vascular. Surgery. 4th ed. Philadelphia: Saunders, 19954). Fig. 1. Type A. Abstract. The normal aorta has three branches from its arch, but variations in this pattern are not uncommon. Our interest was to correlate the docu- mented statistics to the variants observed in our patients. This was.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    DEFF Research Database (Denmark)

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

    2017-01-01

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

  17. [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.

  18. Antenatal evaluation of fetal interrupted aortic arch type B

    Directory of Open Access Journals (Sweden)

    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.

  19. [Thymic carcinoma involving aortic arch; report of a case].

    Science.gov (United States)

    Noriyuki, Toshio; Hamamoto, M; Takazawa, Y; Katoh, K; Hashimoto, M; Kuranishi, F; Nakahara, M; Fukuda, T; Ishizaki, Y; Okuda, H; Akimoto, E; Yonehara, S

    2009-05-01

    Adenocarcinoma of the thymus is a very rare malignant tumor. The standard treatment for advanced thymic carcinoma has not yet been established, and the prognosis is poor. We report a case of thymic carcinoma that involving the aortic arch and the innominate vein. A 78-year-old woman was admitted to our hospital complaining of hoarseness in April 2007. The computed tomography (CT) scan showed an anterior mediastinal tumor contiguous to the aortic arch and the innominate vein with swelling lymphnodes. Microspcopic examinations of specimens obtained by CT-guided needle biopsy revealed poorly differenciated adenocarcinoma. The carcinoembryonic antigen (CEA) level of serum elevated at 54.9 ng/ml. Thymic carcinoma was diagnosed. The chemoradiotherapy [concurrent, carboplatin (CBDCA) + paclitaxel(TXL)-->vinorelbine (NVB), 60 Gy] was performed, but the effect of the therapy was limited. The resection of the tumor with a part of aortic arch and other peripheral tissues was performed in Augast 2007. The postoperative course was uneventful and the CEA level of serum lowered to the normal. She was discharged 30 days after surgery.

  20. Foxc2 in pharyngeal arch mesenchyme is important for aortic arch artery remodelling and ventricular septum formation.

    Science.gov (United States)

    Uddin, Mohammad Khaja Mafij; Kimura, Wataru; Ishikura, Tomoyuki; Koseki, Haruhiko; Yoshida, Nobuaki; Islam, Mohammad Johirul; Amin, Mohammed Badrul; Nakamura, Kasumi; Wu, Yi-Xin; Sato, Eiji; Aoto, Kazushi; Miura, Naoyuki

    2015-01-01

    The forkhead box C2 (Foxc2) protein is a member of the forkhead/winged helix transcription factor family and plays an essential role in cardiovascular development. Previous studies showed that Foxc2 null mouse embryos die during midgestation or just after birth with severe cardiovascular defects, including interruption, coarctation of the aortic arch and ventricular septal defects. These are also seen in human congenital heart disease. However, the tissue specific role of Foxc2 in aortic arch remodelling is not yet fully understood. Here we show that Foxc2 is expressed in a restricted pattern in several cell populations, including the mesenchyme and endothelium of pharyngeal arch arteries, which are important for cardiovascular development. In this study, we use a conditional knockout approach to examine the tissue specific role of Foxc2 in aortic arch remodelling. We demonstrate that mouse embryos lacking Foxc2 in Nkx2.5-expressing mesenchyme and endothelium of pharyngeal arch arteries display aortic arch interruption type B and ventricular septal defects. In contrast, conditional deletion of Foxc2 in Tie2-expressing endothelial cells does not result in aortic arch or ventricular septal defects, but does result in embryonic lethality due to peripheral oedema. Our data therefore provide for a detailed understanding of the role of mesenchymal Foxc2 in aortic arch remodelling and in the development of ventricular septum.

  1. “Open” approach to aortic arch aneurysm repair☆

    Science.gov (United States)

    Al Kindi, Adil H.; Al Kimyani, Nasser; Alameddine, Tarek; Al Abri, Qasim; Balan, Baskaran; Al Sabti, Hilal

    2014-01-01

    Aortic arch aneurysm is a relatively rare entity in cardiac surgery. Repair of such aneurysms, either in isolation or combined with other cardiac procedures, remains a challenging task. The need to produce a relatively bloodless surgical field with circulatory arrest, while at the same time protecting the brain, is the hallmark of this challenge. However, a clear understanding of the topic allows a better and less morbid approach to such a complex surgery. Literature has shown the advantage of selective cerebral perfusion techniques in comparison with only circulatory arrest. Ability to perfuse the brain has allowed circulatory arrest temperatures at moderate hypothermia without the need for deep hypothermia. Even though cannulation site selection appears to be a minor issue, literature has shown that the subclavian/axillary route has the best outcomes and that femoral cannulation should only be reserved for no access patients. Although different techniques for arch anastomosis have been described, we routinely perform the distal first technique as we find it to be less cumbersome and easiest to reproduce. In this review our aim is to outline a systematic approach to aortic arch surgery. Starting with indications for intervention and proceeding with approaches on site of cannulation, approaches to brain protection with hypothermia and selective cerebral perfusion and finally surgical steps in performing the distal and arch vessels anastomosis. PMID:24954988

  2. [Outcomes of endovascular repairing aortic arch disease hybrid with supra-arch debranching procedures].

    Science.gov (United States)

    Wang, Mian; Chang, Guangqi; Yin, Henghui; Yao, Chen; Wang, Jinsong; Wang, Shenming

    2015-11-01

    To summarize the experience of endovascular repairing aortic arch disease hybrid with supra-arch debranching procedures. It was a retrospective study. From January 2002 to December 2014, 42 high risk patients with aortic arch disease were treated by supra-arch debranching hybrid with subsequent endovascular repair in the First Affiliated Hospital of Sun Yat-sen University. There were 39 male and 3 female patients with a mean age of (53±13) years (ranging from 34 to 80 years). Of the 42 patients, 7 were thoracic aortic aneurysm, 20 were Stanford type B aortic dissection and 15 were Stanford type A aortic dissection. After the supra-aortic debranching technique, simultaneous (n=16) or staged (n=26, mean interval (7±3) days) endovascular repair were performed. Fisher exact test was used to compare the in-hospital mortality of ascending aorta based debranching and non-ascending aorta based debranching. Technical success rate was 81.0% (34/42). The overall 30-day complication rate was 31.0% (13/42), including 3 cerebral stroke (7.1%), 8 endoleak (19.0%, including 6 type I endoleak and 2 type II endoleak), 1 circulatory failure, 1 aorto-tracheal fistula. The 30-day mortality was 9.5% (4/42), 2 died of cerebral stroke, 1 died of circulatory failure, 1 died of aorto-tracheal fistula. The in-hospital mortality of ascending aorta based debranching group was obviously higher than that of the non-ascending aorta based debranching group (4/16 vs. 0, P=0.02). The median time of follow-up was 64.8 (2 to 156.9) months. CT scanning was performed at 1, 3 months after surgery and annually thereafter. The overall survival rate was 76.6%. During the follow-up period, there was 4 deaths, and 2 of them were aortic artery related (5.3%). There were 4 de novo complications during the follow-up period, 1 stroke attributed to bypass occlusion was cured by medical treatment, 2 pseudoaneurysm was successfully treated with open surgery, 1 stent-graft induced new distal entry tear was

  3. Distal Transverse Arch to Left Carotid Artery Ratio Helps to Identify Infants With Aortic Arch Hypoplasia.

    Science.gov (United States)

    Swartz, Michael F; Simon, Bartholomew; Atallah-Yunes, Nader; Cholette, Jill M; Orie, Joseph; Gensini, Francisco; Alfieris, George M

    2015-09-01

    Aortic coarctation (CoA) with concomitant aortic arch hypoplasia (AAH) is associated with an increased risk of hypertension after surgical repair. The differentiation of CoA with or without AAH may be critical to delineate the ideal surgical approach that best ameliorates postoperative hypertension. Since 2000, we have defined CoA with AAH when the diameter of the distal transverse aortic arch is equal to or less than the diameter of the left carotid artery. We hypothesized that, based on our definition, aortic tissue from infants having CoA with AAH would demonstrate distinct genetic expression patterns as compared with infants having CoA alone. From 6 infants (AAH, 3; CoA, 3), an Affymetrix 1.0 genome array identified genes in the coarctation/arch region that were differentially expressed between infants having CoA with AAH versus CoA alone. Reverse transcription polymerase chain reaction validated genetic differences from a cohort of 21 infants (CoA with AAH, 10; CoA, 11). To evaluate the clinical outcomes based on our definition of CoA with AAH, we reviewed infants repaired using this algorithm from 2000 to 2010. Microarray data demonstrated genes differentially expressed between groups. Reverse transcription polymerase chain reaction confirmed that CoA with AAH was associated with an increased expression of genes involved in cardiac and vascular development and growth, including hepsin, fibroblast growth factor-18, and T-box 2. The clinical outcomes of 79 infants (AAH, 26; CoA, 53) demonstrated that 90.1% were free of hypertension at 13 years when managed with this surgical strategy. These findings provide evidence that the ratio of the diameter of the distal transverse arch to the left carotid artery may be helpful to identify CoA with AAH and, when used to delineate the surgical approach, may minimize hypertension. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  4. Blunt traumatic aortic injuries of the ascending aorta and aortic arch: a clinical multicentre study.

    Science.gov (United States)

    Mosquera, Victor X; Marini, Milagros; Muñiz, Javier; Gulias, Daniel; Asorey-Veiga, Vanesa; Adrio-Nazar, Belen; Herrera, José M; Pradas-Montilla, Gonzalo; Cuenca, José J

    2013-09-01

    To report the clinical and radiological characteristics, management and outcomes of traumatic ascending aorta and aortic arch injuries. Historic cohort multicentre study including 17 major trauma patients with traumatic aortic injury from January 2000 to January 2011. The most common mechanism of blunt trauma was motor-vehicle crash (47%) followed by motorcycle crash (41%). Patients sustaining traumatic ascending aorta or aortic arch injuries presented a high proportion of myocardial contusion (41%); moderate or greater aortic valve regurgitation (12%); haemopericardium (35%); severe head injuries (65%) and spinal cord injury (23%). The 58.8% of the patients presented a high degree aortic injury (types III and IV). Expected in-hospital mortality was over 50% as defined by mean TRISS 59.7 (SD 38.6) and mean ISS 48.2 (SD 21.6) on admission. Observed in-hospital mortality was 53%. The cause of death was directly related to the ATAI in 45% of cases, head and abdominal injuries being the cause of death in the remaining 55% cases. Long-term survival was 46% at 1 year, 39% at 5 years, and 19% at 10 years. Traumatic aortic injuries of the ascending aorta/arch should be considered in any major thoracic trauma patient presenting cardiac tamponade, aortic valve regurgitation and/or myocardial contusion. These aortic injuries are also associated with a high incidence of neurological injuries, which can be just as lethal as the aortic injury, so treatment priorities should be modulated on an individual basis. Copyright © 2012 Elsevier Ltd. All rights reserved.

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

    International Nuclear Information System (INIS)

    Zhong, Yumin; Zhu, Ming; Sun, Aimin; Li, Yuhua; Jaffe, Richard B.; Gao, Wei

    2007-01-01

    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.)

  6. Asymptomatic Interrupted Aortic Arch, Severe Tricuspid Regurgitation, and Bicuspid Aortic Valve in a 76-Year-Old Woman.

    Science.gov (United States)

    Tajdini, Masih; Sardari, Akram; Forouzannia, Seyed Khalil; Baradaran, Abdolvahab; Hosseini, Seyed Mohammad Reza; Kassaian, Seyed Ebrahim

    2016-10-01

    Interrupted aortic arch is a rare congenital abnormality with a high infancy mortality rate. The principal finding is loss of luminal continuity between the ascending and descending portions of the aorta. Because of the high mortality rate in infancy, interrupted aortic arch is very rare among adults. In this report, we describe the case of a 76-year-old woman with asymptomatic interrupted aortic arch, severe tricuspid regurgitation, and bicuspid aortic valve. To our knowledge, she is the oldest patient ever reported with this possibly unique combination of pathologic conditions. In addition to reporting her case, we review the relevant medical literature.

  7. Atherosclerotic Plaques in the Aortic Arch and Subclinical Cerebrovascular Disease.

    Science.gov (United States)

    Tugcu, Aylin; Jin, Zhezhen; Homma, Shunichi; Elkind, Mitchell S V; Rundek, Tatjana; Yoshita, Mitsuhiro; DeCarli, Charles; Nakanishi, Koki; Shames, Sofia; Wright, Clinton B; Sacco, Ralph L; Di Tullio, Marco R

    2016-11-01

    Aortic arch plaque (AAP) is a risk factor for ischemic stroke, but its association with subclinical cerebrovascular disease is not established. We investigated the association between AAP and subclinical cerebrovascular disease in an elderly stroke-free community-based cohort. The CABL study (Cardiovascular Abnormalities and Brain Lesions) was designed to investigate cardiovascular predictors of silent cerebrovascular disease in the elderly. AAPs were assessed by suprasternal transthoracic echocardiography in 954 participants. Silent brain infarcts and white matter hyperintensity volume (WMHV) were assessed by brain magnetic resonance imaging. The association of AAP thickness with silent brain infarcts and WMHV was evaluated by logistic regression analysis. Mean age was 71.6±9.3 years; 63% were women. AAP was present in 658 (69%) subjects. Silent brain infarcts were detected in 138 participants (14.5%). In multivariate analysis adjusted for potential confounders, AAP thickness and large AAP (≥4 mm in thickness) were significantly associated with the upper quartile of WMHV (WMHV-Q4; odds ratio =1.17; 95% confidence interval, 1.04-1.32; P=0.009 and odds ratio =1.79; 95% confidence interval, 1.40-3.09; P=0.036, respectively), but not with silent brain infarcts (odds ratio =1.08; 95% confidence interval, 0.94-1.23; P=0.265 and odds ratio =1.46; 95% confidence interval, 0.77-2.77; P=0.251, respectively). Aortic arch atherosclerosis was associated with WMHV in a stroke-free community-based elderly cohort. This association was stronger in subjects with large plaques and independent of cardiovascular risk factors. Aortic arch assessment by transthoracic echocardiography may help identify subjects at higher risk of subclinical cerebrovascular disease, who may benefit from aggressive stroke risk factors treatment. © 2016 American Heart Association, Inc.

  8. Geometric Deformations of the Thoracic Aorta and Supra-Aortic Arch Branch Vessels Following Thoracic Endovascular Aortic Repair.

    Science.gov (United States)

    Ullery, Brant W; Suh, Ga-Young; Hirotsu, Kelsey; Zhu, David; Lee, Jason T; Dake, Michael D; Fleischmann, Dominik; Cheng, Christopher P

    2018-04-01

    To utilize 3-D modeling techniques to better characterize geometric deformations of the supra-aortic arch branch vessels and descending thoracic aorta after thoracic endovascular aortic repair. Eighteen patients underwent endovascular repair of either type B aortic dissection (n = 10) or thoracic aortic aneurysm (n = 8). Computed tomography angiography was obtained pre- and postprocedure, and 3-D geometric models of the aorta and supra-aortic branch vessels were constructed. Branch angle of the supra-aortic branch vessels and curvature metrics of the ascending aorta, aortic arch, and stented thoracic aortic lumen were calculated both at pre- and postintervention. The left common carotid artery branch angle was lower than the left subclavian artery angles preintervention ( P Supra-aortic branch vessel angulation remains relatively static when proximal landing zones are distal to the left common carotid artery.

  9. Detecting atheromatous plaques in the aortic arch or supra-aortic arteries for more accurate stroke subtype classification.

    Science.gov (United States)

    Cui, Xiaoyang; Wu, Simiao; Zeng, Quantao; Xiao, Jiahe; Liu, Ming

    2015-02-01

    To investigate the correlations of atheromatous plaques in the aortic arch or supra-aortic arteries with intracranial arterial stenosis and carotid plaques in stroke patients, and to determine whether taking these plaques into account will reduce the proportion of patients in the undetermined etiology group. We prospectively enrolled 308 ischemic stroke patients, whose clinical characteristics and A-S-C-O classifications were compared with analyses of intracranial arteries, carotid arteries, aortic arch, and supra-aortic arteries. 125(40.6%) patients had plaques in the aortic arch or supra-aortic arteries, of which 106 (84.8%) had complex plaques. No correlations were observed between these plaques and carotid plaques ( p = 0.283) or intracranial arterial stenosis ( p = 0.097). After detecting the mobile thrombi in the aortic arch and supra-aortic arteries, the proportion of patients in the atherothrombosis group was increased from 33.8% to 55.5% ( p = 0.00), whereas the proportion of patients in stroke of undetermined etiology group was decreased from 19.2% to 11.0% ( p = 0.00). Examining only the carotid and intracranial arteries may not provide adequate information about large arteries in stroke patients. Therefore, it would be better to include a search for relevant plaques in the aortic arch or supra-aortic arteries in modern stroke workup, for it may lead to more accurate stroke subtype classification and guide secondary prevention.

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

    NARCIS (Netherlands)

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

    2014-01-01

    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

  11. Supra-aortic interventions for endovascular exclusion of the entire aortic arch.

    Science.gov (United States)

    Andrási, Terézia B; Grossmann, Marius; Zenker, Dieter; Danner, Bernhard C; Schöndube, Friedrich A

    2017-07-01

    Our aim was to analyze the outcomes of endovascular exclusion of the entire aortic arch (proximal landing in zone 0, distal landing in zone III or beyond, after Ishimaru) in which complete surgical debranching of the supra-aortic vessels (I), endovascular supra-aortic revascularization (chimney, fenestrated, or branched grafts) with partial surgical debranching (II), or total endovascular supra-aortic revascularization (III) was additionally performed. Publications describing endovascular repair of the aortic arch (2000-2016) were systematically searched and reviewed. From a total of 53 relevant studies including 1853 patients, only 1021 patients undergoing 35 different total aortic arch procedures were found eligible for further evaluation and included in group I, II, or III (429, 190, and 402 patients, respectively). Overall early mortality was higher in group I vs groups II and III (P = .001; 1 - β = 95.6%) but exceeded in group III (18.6%) and group II (14.0%) vs group I (8.0%; P = .044; 1 - β = 57.4%) for diseases involving zone 0. Mortality was higher in all subgroups treated for zone 0 disease compared with corresponding subgroups treated for zone I to zone III disease. The incidence of cerebral ischemic events was increased in groups I and II vs group III (7.5% and 11% vs 1.7%; P = .0001) and correlated with early mortality (R 2  = .20; P = .033). The incidence of type II endoleaks and endovascular reintervention was similar between groups and correlated with each other (R 2  = .37; P = .004). Type Ia endoleak occurred more often in groups II and III than in group I (7.1% and 12.1% vs 5.8%; P = .023) and correlated with midterm mortality (R 2  = .53; P = .005). Retrograde type A dissection was low in all groups, whereas aneurysm growth was higher in group III (2.6%, 4.2%, 10.7%; P = .002), correlating with midterm mortality (R 2  = .311; P = .009). Surgical revision slightly correlated with surgical complications (R 2  = .18; P = .044

  12. RIGHT-SIDED AORTIC ARCH WITH ABERRANT LEFT SUBCLAVIAN ARTERY AND DUPLICATION OF SUPERIOR VENA CAVA

    Directory of Open Access Journals (Sweden)

    Parikhita Hazarika

    2017-08-01

    Full Text Available Right-sided aortic arch is a rare anatomical variant present in about 0.1% of the adult population.1,2 Half of the cases are associated with an aberrant left subclavian artery (0.05%-0.1%. Right-sided aortic arch with aberrant left subclavian artery is less common than left-sided aortic arch with aberrant right subclavian artery (0.5-2.0%.3,4 A rightsided aortic arch is an anatomic variant resulting from persistence of the right fourth aortic arch and involution of the left. It can be associated with an aberrant left subclavian artery arises from Kommerell’s diverticulum. It is usually asymptomatic and diagnosed incidentally during adult age. A 40-year-old male presented with cough and a hump in the back. The patient was evaluated for scoliosis and plain CT thorax was done.

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

    Science.gov (United States)

    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.

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

    International Nuclear Information System (INIS)

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

    1989-01-01

    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.)

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

    Directory of Open Access Journals (Sweden)

    Shirvan Salaminia

    2016-09-01

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

  16. The prevalence of attention-deficit/hyperactivity disorder following neonatal aortic arch repair.

    Science.gov (United States)

    Sistino, Joseph J; Atz, Andrew M; Simpson, Kit N; Ellis, Charles; Ikonomidis, John S; Bradley, Scott M

    2015-04-01

    We sought to determine the prevalence of attention-deficit/hyperactivity disorder in a population of children who underwent neonatal heart surgery involving repair of the aortic arch for Norwood Stage I, interrupted aortic arch, and combined repair of aortic coarctation with ventricular septal defect. Children between the ages of 5 and 16 were surveyed using the ADHD-IV and the Child Heath Questionnaire-50. Classification as attention-deficit/hyperactivity disorder was defined for this study as either a parent-reported diagnosis of attention-deficit/hyperactivity disorder or ADHD-IV inattention score of ⩾93 percentile. Of the 134 surveys, 57 (43%) were returned completed. A total of 25 (44%) children either had a diagnosis of attention-deficit/hyperactivity disorder and/or ADHD-IV inattention score ⩾93 percentile. Eleven of the 13 (85%) children with interrupted aortic arch, 3 of the 7 (42.9%) children with combined coarctation/ventricular septal defect repair, and 9 of the 33 (27.3%) children with hypoplastic left-heart syndrome were classified as having attention-deficit/hyperactivity disorder. Only 7 of the 25 (28%) children received medical treatment for this condition. Quality of life indicators in the Child Heath Questionnaire-50 Questionnaire were highly correlated with the ADHD-IV scores. The risks for the development of attention-deficit/hyperactivity disorder are multifactorial but are significantly increased in this post-surgical population. This study revealed a low treatment rate for attention-deficit/hyperactivity disorder, and a significant impact on the quality of life in these children.

  17. Neonatal aortic arch obstruction due to pedunculated left ventricular foetal myxoma.

    Science.gov (United States)

    Kaulitz, Renate; Haen, Susanne; Sieverding, Ludger

    2015-10-01

    Myxoma in neonatal life are extremely rare. We report a case of a neonate with a pedunculated cardiac tumour arising from the anterolateral left ventricular wall protruding across the left ventricular outflow tract and continuously extending into the distal aortic arch. Surgical removal at 14 days of age via combined transaortic approach and apical ventriculotomy was indicated because of the risk of further compromise of aortic valve function and aortic arch obstruction. Histopathologic examination was consistent with a myxoma.

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

    Science.gov (United States)

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

    2018-02-01

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

  19. Abnormalities of aortic arch shape, central aortic flow dynamics, and distensibility predispose to hypertension after successful repair of aortic coarctation.

    Science.gov (United States)

    Donazzan, Luca; Crepaz, Robert; Stuefer, Josef; Stellin, Giovanni

    2014-10-01

    Systemic hypertension (HT) is a major long-term complication even after successful repair of aortic coarctation (CoA), and many factors are involved in this pathophysiology. To investigate the role of abnormalities in the aortic arch shape, central aortic flow dynamics, and distensibility in developing HT after successful repair of CoA. We selected a group of 26 normotensive patients (mean age 16.9±7.3 years, range 9-32 years) with anatomically successful repair of CoA among 140 patients regularly followed after repair of CoA and analyzed their last clinical and echocardiographic data. Bicycle exercise test and ambulatory blood pressure monitoring (ABPM) were also obtained. Mean age at surgical repair was 3.2±3.9 years (range 10 days-15 years); 12 patients underwent surgical correction during the first year of life. Repair of CoA was performed by end-to-end anastomosis (TT) in 23 patients (extended TT in 6 patients with arch hypoplasia), patch aortoplasty in 2, and subcalvian flap aortoplasty in 1. The postsurgical follow-up was 13.8±7.2 years (range 3.5-29.4 years). The shape of the aortic arch was defined by magnetic resonance imaging (MRI) on this global geometry (normal-gothic-crenel), ratio of the height-transverse diameter (A/T), percentage of residual stenosis, and growth index of the transverse arch segments. Flow mapping by phase-contrast imaging in the ascending and descending aorta was performed in order to measure the systolic waveforms and central aortic distensibility. Twenty normal age-matched patients submitted to the same MRI protocol were used as controls. Six patients were found to have a gothic and 20 a normal aortic arch shape. Patients with gothic aortic arch shape had an increased A/T ratio (0.80±0.07 vs 0.58±0.05, P135 mm Hg on ABPM were higher in the gothic than in the normal arch group. There was a correlation between nocturnal SBP, 24 hours pulse pressure on ABPM in the whole group, and different MRI variables (A/T, distensibility of

  20. Complicated thoraco-abdominal aortic dissection presenting with lower limb ischemia in a patient with bovine arch and arteria lusoria.

    Science.gov (United States)

    Joliat, G-R; Dubuis, C; Déglise, S

    2017-01-01

    We report the case of a 58-year-old man who presented with thoraco-abdominal pain and right lower limb ischemia due to type B aortic dissection. Moreover, the patient was discovered to have several concomitant aortic arch anomalies (bovine arch, arteria lusoria, and left vertebral artery arising from the aortic arch). Taking into account this complex anatomy, emergent femoral exploration with fenestration and thrombectomy was performed. The blood flow to the right lower limb was restored. A few days later the aortic dissection spread proximally, and the aortic arch dilatation enlarged. Before total arch replacement could be performed, the aorta ruptured and the patient unfortunately died.

  1. Equine pericardial roll graft replacement of infected pseudoaneurysm of the aortic arch

    Directory of Open Access Journals (Sweden)

    Kubota Hiroshi

    2012-05-01

    Full Text Available Abstract Resection of the infected aorta, debridement of the surrounding tissue, in situ graft replacement, and omentopexy is the standard procedure for treating infected aortic aneurysms, but the question of which graft material is optimal is still a matter of controversy. We recently treated a patient with an infected thoracic aortic aneurysm. The aneurysm was located in the proximal aortic arch. Because the patients had previously undergone abdominal surgery, the aortic arch were replaced in situ with a branched equine pericardial roll grafts. The patient is alive and well 23 months after the operation.

  2. [Bentall operation combined with total arch replacement and stented elephant trunk implantation for serious Debakey I aortic dissecting aneurysm].

    Science.gov (United States)

    Gu, Tian-Xiang; Wang, Chun; Zhang, Yu-Hai

    2008-12-01

    To summarize the clinical experience of Bentall operation combined with total arch replacement and stented elephant trunk implantation for serious Debakey I aortic dissecting aneurysm. 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 Marfan syndrome in 4 cases, aortic regurgitation in all cases, severely persistent chest pain in 9 cases, acute left heart failure in 8 cases, and cardiac tamponade in 4 cases. Bentall operations combined 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. Urgent surgery underwent in 9 cases. The mean interval between the onset of aortic dissection and the accomplishment of surgery was (41.0 +/- 15.9) hours. Cardiopulmonary bypass time was (191 +/- 26) min, average cross clamp time was (134 +/- 31) min, and average deep hypothermic 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 cases, hemi-paralysis in 1 case, amputation in 1 case, hemorrhage of anastomosis in 1 case, hemorrhage of alimentary tract in 1 case, 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. 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.

  3. Persistent 5th aortic arch--a great pretender: three new covert cases.

    Science.gov (United States)

    Gerlis, L M; Ho, S Y; Anderson, R H; Da Costa, P

    1989-05-01

    Although persistence of the embryonic fifth aortic arch is considered a rare congenital malformation, analysis of the records of the cardiopathological collections of the Brompton and Killingbeck Hospitals reveals 6 examples in approximately 2000 specimens, an incidence amongst our material of 1 in 330. Since our review of the literature reveals only a further 13 described cases, we wonder if the condition may go unrecognized rather than being exceedingly rare. This possibility is further supported by the fact that 3 of our cases were incorrectly interpreted when initially described. In this report, we describe the details of 2 of these specimens and one other case that was not included in our earlier descriptions of this malformation. The first case had the persistent fifth arch as a conduit between the pulmonary and systemic circulations in the setting of aortic atresia with interruption of the aortic arch. The second case demonstrated a double lumen aortic arch, the fifth arch in this instance being an accessory systemic-to-systemic conduit. In the final case, the persistent arch was initially considered to represent an aorto-pulmonary window but review revealed an aortic to pulmonary conduit more in keeping with the presence of a fifth arch. We conclude that the powers of mimicry of this enigmatic structure may account for its apparent rarity.

  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. Variations of the aortic arch - a study on the most common branching patterns

    International Nuclear Information System (INIS)

    Muller, Marguerite; Schmitz, Bernd L; Schick, Melanie; Schloetzer, Wiebke; Pauls, Sandra; Roehrer, Stefan; Kapapa, Thomas

    2011-01-01

    Background: Anatomical variants of the aortic arch and its branching patterns often appear as an incidental finding during routine computed tomography (CT) scanning. These variations can be of relevance when performing angiography or endovascular interventions and may cause symptoms such as dysphagia. Purpose: To analyze common anatomical variations found within the arteries originating from the aortic arch in patients using contrast CT imaging techniques. Material and Methods: A total of 2033 contrast CT scans were analyzed. To obtain a truly representative sample, cases were chosen from different hospital departments without previous knowledge of the patient history. Results: The total percentage of variations within the analyzed patients was 13.3%. In 8.0% a truncus bicaroticus was found. 4.2% of the patients showed a left vertebral artery originating directly from the aortic arch, mostly proximal, and in 1 case distal to the left subclavian artery. In 1.0% we found an aberrant right subclavian artery. We also found a single case of a right descending aortic arch. Conclusion: Variations of the aortic arch and its branching are frequently found, mostly as an incidental finding during routine diagnostic scanning. A contrast-enhanced CT scan is a good method with which to study the aortic arch and its associated branching pattern

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

    Science.gov (United States)

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

    2008-02-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  8. Prenatal diagnosis of 22q11.2 deletion syndrome associated with right aortic arch, left ductus arteriosus, cardiomegaly, and pericardial effusion

    Directory of Open Access Journals (Sweden)

    Yen-Ni Chen

    2016-02-01

    Conclusion: Chromosome abnormalities have been found in patients with RAA. Prenatal diagnosis of RAA with or without intracardiac or extracardiac anomalies should include a diagnosis of 22q11.2 deletion syndrome.

  9. Carotid artery stenting in difficult aortic arch anatomy with or without a new dedicated guiding catheter: preliminary experience.

    Science.gov (United States)

    Barbiero, Giulio; Cognolato, Diego; Casarin, Andrea; Stramanà, Rudi; Galzignan, Elisa; Guarise, Alessandro

    2013-05-01

    To evaluate carotid artery stenting (CAS) procedures with or without a new dedicated guiding catheter in anatomically challenging aortic arches in our experience. We retrospectively reviewed 172 procedures of CAS performed from December 2006 to October 2011 in 159 consecutive patients (100 men, mean age 78 years): 15 patients had type III aortic arch, 13 had a bovine aortic arch, 6 had an acute angle at the origin of the left common carotid artery from the aortic arch, 2 had type III aortic arch with bovine aortic arch, and 1 had a bicarotid trunk with an aberrant right subclavian artery. In this group of difficult anatomy (37 cases), CAS was performed with (13 cases) or without (24 cases) a new dedicated guiding catheter. Mean time of fluoroscopy (16 min vs. 18 min, P guiding catheter group. The new dedicated guiding catheter may be more effective and less risky for CAS in anatomically challenging aortic arches. • Complex anatomy of the aortic arch is not rare • Endovascular carotid artery stenting (CAS) is more difficult when the anatomy is complex • A new dedicated guiding catheter may help CAS when the arch anatomy is complex • The new dedicated guiding catheter may be less risky in complex arches.

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

    Science.gov (United States)

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

    2013-01-01

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

  11. Bovine aortic arch and idiopathic pulmonary artery aneurysm associated with bronchial compression

    Directory of Open Access Journals (Sweden)

    Süleyman Sezai Yıldız

    2015-09-01

    Full Text Available The left common carotid artery originating from the brachiocephalic trunk is termed the bovine aortic arch. Although it is the third most-common normal variant found in 9% humans, the origin of this term remains unclear. Until now, It has not been reported in the literature bovine aortic arch togetherness with pulmonary aneurysm and bronchial compression. Herein, we present a case with bovine aorta arch and pulmonary artery aneurysm associated with bronchial compression, which is incidentally detected by X-ray film. A 56-year-old Caucasian female admitted to the cardiology clinic with complaint of chest pain. Physical examination was unremarkable. Blood biochemistry values and cardiac markers were in normal range. Chest radiography revealed a widened mediastinum and prominent pulmonary conus with no active pulmonary disease. A subsequent transthoracic echocardiography revealed left ventricular hypertrophy, left atrial enlargement (diameter: 41 mm, mild mitral and tricuspid valve insufficiency, dilatation of main pulmonary artery (parasternal short-axis view diameter: 33 mm, normal pulmonary artery pressure and normal left ventricular systolic function. Computed tomography revealed bovine aortic arch associated with pulmonary artery aneurysm (diameter: 53 mm. And left main bronch of trachea was critically squeezed by aortic arch. Aortic and pulmonary vascular anomalies should be considered in patients with chest pain. And, identification with imaging modalities is important for prevention of chronic and irreversible complications.

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

    Science.gov (United States)

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

    2014-09-01

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

  13. Orthotopic branched endovascular aortic arch repair in patients who cannot undergo classical surgery.

    Science.gov (United States)

    Czerny, Martin; Rylski, Bartosz; Morlock, Julia; Schröfel, Holger; Beyersdorf, Friedhelm; Saint Lebes, Bertrand; Meyrignac, Olivier; Mokrane, Fatima; Lescan, Mario; Schlensak, Christian; Hazenberg, Constatijn; Bloemert-Tuin, Trijntje; Braithwaite, Sue; van Herwaarden, Joost; Rousseau, Herve

    2018-01-16

    Our goal was to assess the results after orthotopic branched endovascular aortic arch repair using a new double-branch endoprosthesis in patients with thoracic aortic disease affecting the aortic arch who cannot undergo classical surgery. Within a 4-year period, 15 patients with thoracic aortic disease affecting the aortic arch were treated with the Bolton Relay plus double-branch endoprosthesis (Bolton Medical, Sunrise, FL, USA). We assessed clinical outcome, occurrence of endoleaks and the need for secondary interventions. The median logistic EuroSCORE I level was 13.6 (4.2; 22.8). The in-hospital mortality rate was 6.7%. A disabling stroke was observed in 1 (6.7%) patient, whereas non-disabling strokes occurred in 2 (13.3%) patients. Type I and III endoleaks occurred in 6.7%. The median follow-up period was 263 (1st quartile 84; 2nd quartile 564) days. Four patients died during the follow-up period. Aortic-related survival was 100%. Orthotopic branched endovascular aortic arch repair using the Bolton Relay Plus double-branch endoprosthesis is a safe and feasible technique enriching the armamentarium to treat patients with thoracic aortic disease who cannot undergo classical surgery. Aortic-related survival is excellent, and the occurrence of disabling stroke and endoleaks warranting treatment is low. Further studies are needed to assess the long-term durability of this new method. © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  14. Activation of coagulation and hyperfibrinolysis in patients with aortic arch atheromatosis (Aortic AA) as a risk factor for cerebral ischemia.

    Science.gov (United States)

    Siemens, Hans-Joachim G; Mirau, Wladimir; Brueckner, Sabine; Jahn, Juergen; Roth-Isigkeit, Angela; Gutsche, Sven; Mitusch, Rolf; Sheikhzadeh, Abdolhamid

    2005-04-01

    In patients with cerebral ischemia, a frequent finding is atheromatous plaques in the ascending aorta and the aortic arch. Since we were able to demonstrate that patients with atrial fibrillation have an increased coagulatory activity, we wanted to evaluate a potential systemic activation of the coagulatory system in patients with aortic arch atheromatosis (Aortic AA). In 134 consecutive patients, we determined several parameters of the coagulatory and fibrinolytic systems as well as several thrombophilia risk factors and compared the results with 134 age- and sex-matched healthy controls. In 90 of the 134 patients, transesophageal echocardiography showed Aortic AA, and in the remaining 44 patients, there were no aortic findings. The Aortic AA group showed higher concentrations of thrombin-antithrombin (TAT) and plasmin-antiplasmin complexes (PAP). Further division into 4 subgroups of different severity (grade I: no plaques; grade II: plaques 2-5 mm, grade III: plaques > 5 mm, grade IV: mobile plaques), revealed increasing concentrations of fibrinogen, D-dimers and tissue-type plasminogen activator. The grade IV-group displayed the highest values in comparison to all other groups. In conclusion, Aortic AA as such is a risk factor for cerebral ischemia. It causes a systemically detectable activation of coagulation which substantially exceeds the values for controls. This observation is in accordance with our findings in patients with atrial fibrillation.

  15. GROWTH OF THE HYPOPLASTIC AORTIC-ARCH AFTER SIMPLE COARCTATION RESECTION AND END-TO-END ANASTOMOSIS

    NARCIS (Netherlands)

    BROUWER, MHJ; CROMMEDIJKHUIS, AH; EBELS, T; EIJGELAAR, A

    Surgical treatment of a hypoplastic aortic arch associated with an aortic coarctation is controversial. The controversy concerns the claimed need to surgically enlarge the diameter of the hypoplastic arch, in addition to resection and end-to-end anastomosis. The purpose of this prospective study is

  16. The surgical anatomy of the left ventricular outflow tract in hearts with ventricular septal defect and aortic arch obstruction

    NARCIS (Netherlands)

    Shiokawa, Y.; Becker, A. E.

    1998-01-01

    Profound understanding of the left ventricular outflow tract (LVOT) anatomy is crucial to improve surgical results in patients with aortic arch obstruction, ventricular septal defect, and subaortic stenosis. We studied the morphology of the LVOT in 32 postmortem hearts with aortic arch obstruction

  17. Anatomic variations of the branches of the aortic arch in a Peruvian population.

    Science.gov (United States)

    Huapaya, Julio Arturo; Chávez-Trujillo, Kristhy; Trelles, Miguel; Dueñas Carbajal, Roy; Ferrandiz Espadin, Renato

    2015-07-31

    Previous publications from two countries in South America found one anatomical variation not previously reported in the rest of the world, which in turn give some clues with regard to a racial difference. The objective of the present study is to describe variations in the anatomical distribution of the branches of the aortic arch in a Peruvian population. To describe variations in the anatomical distribution of the branches of the aortic arch in a Peruvian population. A descriptive study of patients who underwent a tomography angiography of the aorta was performed. We analyzed the reports that showed the description of the variations of the branches of the aortic arch based on the eight types currently described in the literature. From 361 analyzed reports, 282 patients (78.12%) had a normal aortic arch configuration (type I; aortic arch gives rise to the brachiocephalic trunk, left common carotid and left subclavian arteries); followed by type II (left common carotid artery as a branch of the aorta) with 41 patients (11.36%); and type IX (common ostium for the brachiocephalic trunk and the left common carotid artery) with 25 patients (6.93%). The latter and two other types are new variations. Aortic Arch Type I, Type II and Type IX were the most frequent variations in this Peruvian study. Additionally, we also found two more new types that have not been previously described in the literature. Further investigation regarding these variations is needed in order to assess a racial factor in South America and possible relationships with clinical or surgical events.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

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

    2014-04-01

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

  20. Prenatal ultrasonic diagnosis and differential diagnosis of isolated right aortic arch with mirror-image branching.

    Science.gov (United States)

    Gao, Junxue; Zhu, Jiaan; Pei, Qiuyan; Li, Jianguo

    2017-05-01

    This study sought to evaluate the fetal echocardiography features of isolated right aortic arch (RAA) with mirror-image branching and to improve the rate and accuracy of prenatal diagnosis of this condition. We reviewed fetal echocardiograms from all cases of isolated RAA with mirror-image branching diagnosed at our institution between August 2012 and December 2015 and classified these cases into normal and abnormal types of ductus arteriosus based on the course of the arterial duct arch. We confirmed the diagnoses by postnatal echocardiography. A total of 11 cases of isolated RAA with mirror-image branching, with the left ductus and the descending aorta located on the left side of the spine, were diagnosed using fetal echocardiography. Ten cases involved normal ductus arteriosus, with the left ductus connecting the left pulmonary artery to the descending aorta, five of which were referred to our institution for suspicions of double aortic aorta. 1 case involved abnormal ductus arteriosus, with the left ductus connecting the left pulmonary artery to the left innominate artery. RAA with mirror-image branching can be detected via fetal echocardiography, which can reveal the relationship between of the aortic arch and the trachea and can enable the identification of the course of brachiocephalic branching. The identification of isolated RAA with mirror-image branching is crucial for distinguishing this condition from other types of aortic arch anomalies, particularly double aortic aorta, which can have a rather different prognosis.

  1. 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.

  2. A new technique for interrupted aortic arch repair: the Neville tube.

    Science.gov (United States)

    Bergoënd, Eric; Bouissou, Antoine; Paoli, Florent; Roullet-Renoleau, Nicolas; Duchalais, Alain; Neville, Paul

    2010-10-01

    We have developed a new technique for interrupted aortic arch repair in which the pulmonary artery anterior wall is cut off and tailored so as to re-establish aortic continuity with an autologous tube. We are describing this method herein, with an 8-year follow-up of the first patient. Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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

    International Nuclear Information System (INIS)

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

    1995-01-01

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

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

    Science.gov (United States)

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

    2006-09-20

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

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

    Directory of Open Access Journals (Sweden)

    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.

  6. Abnormal pulmonary vein drainage in upper right lobe associated with double aortic arch : magnetic resonance angiography

    International Nuclear Information System (INIS)

    Busto, M.; Dolz, J.L.; Capdevilla, A.; Castanon, M.; Mulet, J.

    1997-01-01

    We present the magnetic resonance (MR) and magnetic resonance angiography (MRA) findings in a case of abnormal pulmonary vein drainage from upper right lobe to superior vena cava, associated with double aortic arch, in a six-month-old boy. (Author) 9 refs

  7. [Antegrade unilateral perfusion of the brain through the brachiocephalic trunk in operations on the aortic arch].

    Science.gov (United States)

    Kozlov, B N; Panfilov, D S; Kuznetsov, M S; Ponomarenko, I V; Nasrashvili, G G; Shipulin, V M

    2016-01-01

    Presented herein is a technique of unilateral antegrade perfusion of the brain in operations on the aortic arch. The method makes it possible to perform both systemic artificial circulation and adequate physiological perfusion of the brain, promoting minimization of the number of neurological complications.

  8. Traumatic partial avulsion of a single right subclavian artery from the aortic arch and definitive repair.

    Science.gov (United States)

    Kapetanakis, Emmanouil I; Sears-Rogan, Pamela; Young, Richard S; Kanda, Louis T; Ellis, Jennifer L

    2006-01-01

    Blunt injury to the right subclavian artery is a rare complication of severe deceleration trauma often associated with significant morbidity and mortality. We describe an atypical presentation in a patient who sustained a traumatic avulsion of his right subclavian artery arising off the aortic arch. An interposition graft was used to restore the continuity of the artery to the ascending thoracic aorta.

  9. A review of the surgical management of right-sided aortic arch aneurysms.

    Science.gov (United States)

    Barr, James G; Sepehripour, Amir H; Jarral, Omar A; Tsipas, Pantelis; Kokotsakis, John; Kourliouros, Antonios; Athanasiou, Thanos

    2016-07-01

    Aneurysms and dissections of the right-sided aortic arch are rare and published data are limited to a few case reports and small series. The optimal treatment strategy of this entity and the challenges associated with their management are not yet fully investigated and conclusive. We performed a systematic review of the literature to identify all patients who underwent surgical or endovascular intervention for right aortic arch aneurysms or dissections. The search was limited to the articles published only in English. We focused on presentation and critically assessed different management strategies and outcomes. We identified 74 studies that reported 99 patients undergoing surgical or endovascular intervention for a right aortic arch aneurysm or dissection. The median age was 61 years. The commonest presenting symptoms were chest or back pain and dysphagia. Eighty-eight patients had an aberrant left subclavian artery with only 11 patients having the mirror image variant of a right aortic arch. The commonest pathology was aneurysm arising from a Kommerell's diverticulum occurring in over 50% of the patients. Twenty-eight patients had dissections, 19 of these were Type B and 9 were Type A. Eighty-one patients had elective operations while 18 had emergency procedures. Sixty-seven patients underwent surgical treatment, 20 patients had hybrid surgical and endovascular procedures and 12 had totally endovascular procedure. There were 5 deaths, 4 of which were in patients undergoing emergency surgery and none in the endovascular repair group. Aneurysms and dissections of a right-sided aortic arch are rare. Advances in endovascular treatment and hybrid surgical and endovascular management are making this rare pathology amenable to these approaches and may confer improved outcomes compared with conventional extensive repair techniques. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights

  10. Right Aortic Arch and Kommerell's Diverticulum Repaired without Reconstruction of Aberrant Left Subclavian Artery.

    Science.gov (United States)

    Osawa, Hiroshi; Shinohara, Daisuke; Orii, Kouan; Hosaka, Shigeru; Fukuda, Shoji; Akashi, Okihiko; Furukawa, Hiroshi

    2013-01-01

    Right aortic arch with Kommerell's diverticulum is a very rare situation. Surgical treatment is recommended for symptomatic patients or asymptomatic patients with a large diverticulum. However planning the strategy of operation is difficult without a 3D imaging. We report a case of a 57-year-old man with right aortic arch, Kommerell's diverticulum, and aberrant left subclavian artery. After a 3D-CT imaging, the patient underwent descending aortic replacement without reconstruction of aberrant left subclavian artery. After operation, there was no signs or symptoms of ischemia of the left arm. If the reconstruction of the aberrant subclavian artery was too difficult, closing its orifice is an acceptable decision. It has been found advantageous because of a decrease blood loss and a shorter cardiopulmonary bypass duration. If an ischemia of the arm is noticed, additional reconstruction will have to be considered. 3D-CT imaging was very useful to have a proper orientation and plan for the operative strategy.

  11. A Right-sided Aortic Arch with Kommerell's Diverticulum of the Aberrant Left Subclavian Artery Presenting with Syncope

    Directory of Open Access Journals (Sweden)

    Ming-Hsun Yang

    2009-05-01

    Full Text Available A right-sided aortic arch with an aneurysm of the aberrant subclavian artery is a rare disease. We report a case of Kommerell's diverticulum of an aberrant left subclavian artery in a patient with a right-sided aortic arch. Fewer than 50 cases have been reported in the literature. A number of operative strategies are described. Right thoracotomy provides good exposure and avoids the morbidity associated with bilateral thoracotomy or sternotomy and thoracotomy. In our patient with symptoms of dysphagia, syncope, and left subclavian steal syndrome, a left thoracotomy was used. The repair was accomplished by division of a left ligamentum arteriosum, obliteration of the Kommerell's aneurysm, and an aorto-subclavian bypass. Postoperative complications included left vocal cord palsy and Horner's syndrome. Hoarseness and left ptosis recovered spontaneously 3 months after surgery, and the patient remained symptom-free at the 1-year follow-up. We believe a left thoracotomy for direct repair of Kommerell's diverticulum is a simple and safe method without the increased morbidity found in other procedures.

  12. Angular (Gothic) aortic arch leads to enhanced systolic wave reflection, central aortic stiffness, and increased left ventricular mass late after aortic coarctation repair: evaluation with magnetic resonance flow mapping.

    Science.gov (United States)

    Ou, Phalla; Celermajer, David S; Raisky, Olivier; Jolivet, Odile; Buyens, Fanny; Herment, Alain; Sidi, Daniel; Bonnet, Damien; Mousseaux, Elie

    2008-01-01

    We sought to investigate the mechanism whereby a particular deformity of the aortic arch, an angulated Gothic shape, might lead to hypertension late after anatomically successful repair of aortic coarctation. Fifty-five normotensive patients with anatomically successful repair of aortic coarctation and either a Gothic (angulated) or a Romanesque (smooth and rounded) arch were studied with magnetic resonance angiography and flow mapping in both the ascending and descending aortas. Systolic waveforms, central aortic stiffness, and pulse velocity were measured. We hypothesized that arch angulation would result in enhanced systolic wave reflection with loss of energy across the aortic arch, as well as increased central aortic stiffness. Twenty patients were found to have a Gothic, and 35 a Romanesque, arch. Patients with a Gothic arch showed markedly augmented systolic wave reflection (12 +/- 6 vs 5 +/- 0.3 mL, P Gothic arch (5.6 +/- 1.1 vs 4.1 +/- 1 m/s, P Gothic aortic arch is associated with increased systolic wave reflection, as well as increased central aortic stiffness and left ventricular mass index. These findings explain (at least in part) the association between this pattern of arch geometry and late hypertension at rest and on exercise in subjects after coarctation repair.

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

    Directory of Open Access Journals (Sweden)

    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.

  14. Morphometric changes in the aortic arch with advancing age in fetal to mature thoroughbred horses.

    Science.gov (United States)

    Endoh, Chihiro; Matsuda, Kazuya; Okamoto, Minoru; Tsunoda, Nobuo; Taniyama, Hiroyuki

    2017-03-28

    Aortic rupture is a well recognized cause of sudden death in thoroughbred horses. Some microscopic lesions, such as those caused by cystic medial necrosis and medionecrosis, can lead to aortic rupture. However, these microscopic lesions are also observed in normal horses. On the other hand, a previous study of aortic rupture suggested that underlying elastin and collagen deposition disorders might be associated with aortic rupture. Therefore, the purpose of this study was to compare the structural components of the tunica media of the aortic arch, which is composed of elastin, collagen, smooth muscle cells and mucopolysaccharides (MPS), in fetal to mature thoroughbred horses. The percentage area of elastin was greatest in the young horses and subsequently decreased with aging. The percentage area of collagen increased with aging, and the elderly horses (aged ≥20) exhibited significantly higher percentage areas of collagen than the young horses. The percentage area of smooth muscle cells did not change with age. The percentage area of MPS was inversely proportional to the percentage area of elastin. The fetuses exhibited a markedly larger percentage area of MPS than the mature horses. We concluded that the medial changes seen in the aortic arch, which included a reduction in the amount of elastin and increases in the amounts of collagen and MPS, were age-related variations.

  15. Retrosternal friction-induced late disruption of the anastomotic site between Bentall's valved conduit and an aortic arch graft: report of a case.

    Science.gov (United States)

    Fukada, Johji; Morishita, Kiyofumi; Kawaharada, Nobuyoshi; Kurimoto, Yoshihiko; Muraki, Satoshi; Satsu, Takuma; Abe, Tomio

    2003-01-01

    We report a case of late mediastinal false aneurysm originating from disruption of the suture line between synthetic vascular grafts for aortic root and total aortic arch replacements. This aneurysm developed without any infection in a patient with Marfan's syndrome. To our knowledge, this event has never been reported before. The only possible cause of this disruption was that the monofilament suture was broken by continuous friction between the pointed anastomotic line and the sternum since the operation. The treatment options for this unusual event after extended synthetic graft replacement are discussed.

  16. Selective Cerebro-Myocardial Perfusion in Complex Neonatal Aortic Arch Pathology: Midterm Results.

    Science.gov (United States)

    Hoxha, Stiljan; Abbasciano, Riccardo Giuseppe; Sandrini, Camilla; Rossetti, Lucia; Menon, Tiziano; Barozzi, Luca; Linardi, Daniele; Rungatscher, Alessio; Faggian, Giuseppe; Luciani, Giovanni Battista

    2018-03-06

    Aortic arch repair in newborns and infants has traditionally been accomplished using a period of deep hypothermic circulatory arrest. To reduce neurologic and cardiac dysfunction related to circulatory arrest and myocardial ischemia during complex aortic arch surgery, an alternative and novel strategy for cerebro-myocardial protection was recently developed, where regional low-flow perfusion is combined with controlled and independent coronary perfusion. The aim of the present retrospective study was to assess short-term and mid-term results of selective and independent cerebro-myocardial perfusion in neonatal aortic arch surgery. From April 2008 to August 2015, 28 consecutive neonates underwent aortic arch surgery under cerebro-myocardial perfusion. There were 17 male and 11 female, with median age of 15 days (3-30 days) and median body weight of 3 kg (1.6-4.2 kg), 9 (32%) of whom with low body weight (cerebro-myocardial perfusion was 30 ± 11 min (15-69 min). Renal dysfunction, requiring a period of peritoneal dialysis was observed in 10 (36%) patients, while liver dysfunction was noted only in 3 (11%). There were three (11%) early and two late deaths during a median follow-up of 2.9 years (range 6 months-7.7 years), with an actuarial survival of 82% at 7 years. At latest follow-up, no patient showed signs of cardiac or neurologic dysfunction. The present experience shows that a strategy of selective and independent cerebro-myocardial perfusion is safe, versatile, and feasible in high-risk neonates with complex congenital arch pathology. Encouraging outcomes were noted in terms of cardiac and neurological function, with limited end-organ morbidity. © 2018 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

  17. Incidental Finding of a Large Mobile Aortic Arch Mass during Conventional Angiography

    Directory of Open Access Journals (Sweden)

    Hassan Aghajani

    2018-02-01

    Full Text Available Thromboembolism occurs commonly in general practice and leads to significant health burden. Apart from cardiac sources, aortic atherosclerotic plaques contribute considerably to thromboembolism. A 63-year-old diabetic hypertensive woman referred to our center due to exertional chest pain unresponsive to optimal medical therapy and underwent coronary angiography. Owing to resistance during guide-wire advancement, an aortography was performed. Aortic arch injection demonstrated a large suspended mass distal to the left subclavian artery with free movement in the descending thoracic aorta. Echocardiography revealed widespread atherosclerotic changes in the aortic arch with a large hypermobile mass. Dual-source multi-slice (2 × 128:256 computed tomography angiography of the whole aorta revealed a large floating mass (in favor of a thrombus in the distal portion of the arch. The patient underwent coronary artery bypass grafting due to severe coronary artery disease. The intra-aortic mass, which was actually a large atherosclerotic plaque, was resected at the same session. She was discharged uneventfully and during a 1-year follow-up, she had no embolic events.

  18. Surgical repair of distal arch psendoaneurysm from ruptured penetrating aortic ulcer with the frozen elephant trunk technique.

    Science.gov (United States)

    Kokotsakis, John; Tassopoulos, Dimitrios; Ttofi, Jacob; Harling, Leanne; Ashrafian, Hutan; Velissarios, Konstantinos; Kratimenos, Theodore; Anagnostou, Stratos; Athanasiou, Thanos

    2014-04-05

    Ruptured Penetrating Ulcer and aortic arch pseudo-aneurysm is a rare condition but one which carries a high risk of rupture. We report the case of a 74-year-old man with aortic arch pseudo-aneurysm, in which a Frozen Elephant Trunk procedure was successfully performed. There were no postoperative complications at 6 months follow-up. The Computed Tomography Angiogram demonstrated thrombus formation in the pseudo-aneurysm lumen, with no endoleak on the stented part of the descending thoracic aorta and complete patency of all branches of aortic arch. This case demonstrates that the Frozen Elephant Trunk technique may be the treatment of choice when treating such complex aortic arch lesions provided there is no absolute contraindication to radical surgical intervention. However, long-term clinical efficacy and safety have yet to be confirmed.

  19. [Metabolic syndrome and aortic stiffness].

    Science.gov (United States)

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

    2010-09-01

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

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

    Science.gov (United States)

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

    2008-01-01

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

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

    Science.gov (United States)

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

    2008-06-01

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

  2. 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.)

  3. Endovascular Retrieval of Entrapped Elephant Trunk Graft During Complex Hybrid Aortic Arch Repair

    Energy Technology Data Exchange (ETDEWEB)

    Damodharan, Karthikeyan, E-mail: drdkarthik@hotmail.com [Singapore General Hospital, Department of Diagnostic Radiology (Singapore); Chao, Victor T. T., E-mail: victor.chao.t.t@singhealth.com.sg [National Heart Centre, Department of Cardiothoracic Surgery (Singapore); Tay, Kiang Hiong, E-mail: tay.kiang.hiong@singhealth.com.sg [Singapore General Hospital, Department of Diagnostic Radiology (Singapore)

    2016-12-15

    Entrapment of the elephant trunk graft within the false lumen is a rare complication of surgical repair of an aortic dissection. This is normally retrieved by emergent open surgery. We describe a technique of endovascular retrieval of the dislodged graft, during hybrid aortic arch repair. The elephant trunk was cannulated through and through from a femoral access and the free end of the wire was snared and retrieved from a brachial access. The wire was externalised from both accesses and was used to reposition the graft into the true lumen using a body flossing technique.

  4. MRI-based multiscale models for the hemodynamic and structural evaluation of surgically reconstructed aortic arches

    DEFF Research Database (Denmark)

    Pittaccio, S; Migliavacca, F; Balossino, R

    2007-01-01

    The surgical reconstruction of the aortic arch is necessary in pediatric patients suffering from different types of congenital heart malformations, in particular, coarctation of the aorta. Among the reconstruction techniques used in surgical practice end-to-end anastomosis (E/E), Gore-tex graft...... interposition (GGI) and Gore-tex patch graft aortoplasty (GPGA) are compared in this study with a control model, employing a computational fluid-structure-interaction scheme. This study analyzes the impact of introducing synthetic materials on aortic hemodynamics and wall mechanics. Three-dimensional (3D...

  5. 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).

  6. Computational hemodynamic optimization predicts dominant aortic arch selection is driven by embryonic outflow tract orientation in the chick embryo.

    Science.gov (United States)

    Kowalski, William J; Teslovich, Nikola C; Dur, Onur; Keller, Bradley B; Pekkan, Kerem

    2012-09-01

    In the early embryo, a series of symmetric, paired vessels, the aortic arches, surround the foregut and distribute cardiac output to the growing embryo and fetus. During embryonic development, the arch vessels undergo large-scale asymmetric morphogenesis to form species-specific adult great vessel patterns. These transformations occur within a dynamic biomechanical environment, which can play an important role in the development of normal arch configurations or the aberrant arch morphologies associated with congenital cardiac defects. Arrested migration and rotation of the embryonic outflow tract during late stages of cardiac looping has been shown to produce both outflow tract and several arch abnormalities. Here, we investigate how changes in flow distribution due to a perturbation in the angular orientation of the embryonic outflow tract impact the morphogenesis and growth of the aortic arches. Using a combination of in vivo arch morphometry with fluorescent dye injection and hemodynamics-driven bioengineering optimization-based vascular growth modeling, we demonstrate that outflow tract orientation significantly changes during development and that the associated changes in hemodynamic load can dramatically influence downstream aortic arch patterning. Optimization reveals that balancing energy expenditure with diffusive capacity leads to multiple arch vessel patterns as seen in the embryo, while minimizing energy alone led to the single arch configuration seen in the mature arch of aorta. Our model further shows the critical importance of the orientation of the outflow tract in dictating morphogenesis to the adult single arch and accurately predicts arch IV as the dominant mature arch of aorta. These results support the hypothesis that abnormal positioning of the outflow tract during early cardiac morphogenesis may lead to congenital defects of the great vessels due to altered hemodynamic loading.

  7. Combined model-based segmentation and elastic registration for accurate quantification of the aortic arch.

    Science.gov (United States)

    Biesdorf, Andreas; Rohr, Karl; von Tengg-Kobligk, Hendrik; Wörz, Stefan

    2010-01-01

    Accurate quantification of the morphology of vessels is important for diagnosis and treatment of cardiovascular diseases. We introduce a new approach for the quantification of the aortic arch morphology that combines 3D model-based segmentation with elastic image registration. The performance of the approach has been evaluated using 3D synthetic images and clinically relevant 3D CTA images including pathologies. We also performed a comparison with a previous approach.

  8. 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.

  9. Abnormal innominate vein and right aortic arch, an uncommon association that should be taken into account

    International Nuclear Information System (INIS)

    Catala, J.; Martin, C.

    2000-01-01

    A case of abnormal innominate vein associated with right aortic arch and aberrant left subclavian artery is presented. It was an incidental finding during the radiological study of an 8-year old boy suspected of having tuberculosis. The authors review the different etiological theories, radiological features and diagnostic impact of this uncommon venous malformation, as well as its relationship to other cardiovascular anomalies. (Author) 9 refs

  10. 3-D quantification of the aortic arch morphology in 3-D CTA data for endovascular aortic repair.

    Science.gov (United States)

    Wörz, Stefan; von Tengg-Kobligk, Hendrik; Henninger, Verena; Rengier, Fabian; Schumacher, Hardy; Böckler, Dittmar; Kauczor, Hans-Ulrich; Rohr, Karl

    2010-10-01

    We introduce a new model-based approach for the segmentation and quantification of the aortic arch morphology in 3-D computed tomography angiography (CTA) data for thoracic endovascular aortic repair (TEVAR). The approach is based on a model-fitting scheme using a 3-D analytic intensity model for thick vessels in conjunction with a two-step refinement procedure, and allows us to accurately quantify the morphology of the aortic arch. Based on the fitting results, we additionally compute the (local) 3-D vessel curvature and torsion as well as the relevant lengths not only along the 3-D centerline, but particularly also along the inner and outer contour. These measurements are important for preoperative planning in TEVAR applications. We have validated our approach based on 3-D synthetic as well as 3-D MR phantom images. Moreover, we have successfully applied our approach using 3-D CTA datasets of the human thorax and have compared the results with ground truth obtained by a radiologist. We have also performed a quantitative comparison with a commercial vascular analysis software.

  11. Transcarotid Artery Endovascular Reconstruction of the Aortic Arch by Modified Bifurcated Stent Graft for Stanford Type A Dissection

    Directory of Open Access Journals (Sweden)

    Wei Guo

    2007-10-01

    Full Text Available A 40-year-old man with Stanford type B dissection underwent his first endovascular repair (EVAR in April 2004 by Talent thoracic stent graft. He had an uncomplicated recovery and maintained good blood pressure control. However, a new retrograde dissection appeared in September 2004. The new dissection involved his aortic arch and ascending thoracic aorta to the opening of the coronary arteries. To reconstruct the aortic arch, bypasses between the right common carotid artery (RCCA, left common carotid artery and left subclavian artery were performed before endovascular repair. A modified bifurcated Talent stent graft was deployed from the RCCA to the ascending thoracic aorta with a long limb in the innominate artery and a short limb in the aortic arch. A further two pieces of graft were deployed via the common femoral artery. The ascending thoracic aorta and aortic arch were reconstructed completely by the bifurcated stent graft. The final angiography confirmed that there was good stent graft configuration, normal blood flow, and stable haemodynamics. No endoleak or other major complications were encountered. This result indicated that it is possible to reconstruct the aortic arch with a bifurcated stent graft and could be a new endovascular repair model for complex thoracic aortic aneurysm and dissection.

  12. Aortic arch atherosclerosis in patients with severe aortic stenosis can be argued by greater day-by-day blood pressure variability.

    Science.gov (United States)

    Iwata, Shinichi; Sugioka, Kenichi; Fujita, Suwako; Ito, Asahiro; Matsumura, Yoshiki; Hanatani, Akihisa; Takagi, Masahiko; Di Tullio, Marco R; Homma, Shunichi; Yoshiyama, Minoru

    2015-07-01

    Although it is well known that the prevalence of aortic arch plaques, one of the risk factors for ischemic stroke, is high in patients with severe aortic stenosis, the underlying mechanisms are not well understood. Increased day-by-day blood pressure (BP) variability is also known to be associated with stroke; however, little is known on the association between day-by-bay BP variability and aortic arch atherosclerosis in patients with aortic stenosis. Our objective was to clarify the association between day-by-day BP variables (average values and variability) and aortic arch atherosclerosis in patients with severe aortic stenosis. The study population consisted of 104 consecutive patients (mean age 75 ± 8 years) with severe aortic stenosis who were scheduled for aortic valve replacement. BP was measured in the morning in at least 4 consecutive days (mean 6.8 days) prior to the day of surgery. Large (≥4 mm), ulcerated, or mobile plaques were defined as complex plaques using transesophageal echocardiography. Cigarette smoking and all systolic BP variables were associated with the presence of complex plaques (p < 0.05), whereas diastolic BP variables were not. Multiple regression analysis indicated that day-by-day mean systolic BP and day-by-day systolic BP variability remained independently associated with the presence of complex plaques (p < 0.05) after adjustment for age, male sex, cigarette smoking, hypertension, hypercholesterolemia, and diabetes mellitus. These findings suggest that higher day-by-day mean systolic BP and day-by-day systolic BP variability are associated with complex plaques in the aortic arch and consequently stroke risk in patients with aortic stenosis. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  13. Validation of numerical simulation methods in aortic arch using 4D Flow MRI.

    Science.gov (United States)

    Miyazaki, Shohei; Itatani, Keiichi; Furusawa, Toyoki; Nishino, Teruyasu; Sugiyama, Masataka; Takehara, Yasuo; Yasukochi, Satoshi

    2017-08-01

    Computational fluid dynamics (CFD) are the gold standard in studying blood flow dynamics. However, CFD results are dependent on the boundary conditions and the computation model. The purpose of this study was to validate CFD methods using comparison with actual measurements of the blood flow vector obtained with four-dimensional (4D) flow magnetic resonance imaging (MRI). 4D Flow MRI was performed on a healthy adult and a child with double-aortic arch. The aortic lumen was segmented to visualize the blood flow. The CFD analyses were performed for the same geometries based on three turbulent models: laminar, large eddy simulation (LES), and the renormalization group k-ε model (RNG k-ε). The flow-velocity vector components, namely the wall shear stress (WSS) and flow energy loss (EL), of the MRI and CFD results were compared. The flow rate of the MRI results was underestimated in small vessels, including the neck vessels. Spiral flow in the ascending aorta caused by the left ventricular twist was observed by MRI. Secondary flow distal to the aortic arch was well realized in both CFD and MRI. The average correlation coefficients of the velocity vector components of MRI and CFD for the child were the highest for the RNG k-ε model (0.530 in ascending aorta, 0.768 in the aortic arch, 0.584 in the descending aorta). The WSS and EL values of MRI were less than half of those of CFD, but the WSS distribution patterns were quite similar. The WSS and EL estimates were higher in RNG k-ε and LES than in the laminar model because of eddy viscosity. The CFD computation realized accurate flow distal to the aortic arch, and the WSS distribution was well simulated compared to actual measurement using 4D Flow MRI. However, the helical flow was not simulated in the ascending aorta. The accuracy was enhanced by using the turbulence model, and the RNG k-ε model showed the highest correlation with 4D Flow MRI.

  14. [Hybrid surgical intervention in a patient with an aortic arch aneurysm and coronary artery disease].

    Science.gov (United States)

    Charchan, E R; Abugov, S A; Puretsky, M V; Kim, S Yu; Skvortsov, A A; Khachatryan, Z R

    2015-01-01

    Presented herein is a clinical case report regarding the use of hybrid technology in surgical treatment of a patient with an aneurysm of the distal portion of the aortic arch and coronary artery disease. The patient underwent a hybrid operation, i.e. debranching of the aortic arch branches, exoprosthetic repair of the ascending aorta, autovenous prosthetic coronary bypass grafting of the branch of the blunt edge of the anterior interventricular artery, stenting of the ascending portion, arch and descending portion of the aorta (stent graft "Medtronic Valiant"). In doing so, we used a non-standard approach to connecting the artificial circulation unit and to choosing the place for establishing proximal anastomoses of autovenous coronary bypass grafts. The early postoperative period was complicated by the development of respiratory insufficiency requiring continuation artificial pulmonary ventilation. The duration of the hospital stay of the patient amounted to 15 days. The check-up multispiral computed tomography showed normal functioning of the reconstruction zones, the stent graft is expanded, with no leak observed. The conclusion was made that hybrid interventions may be considered as an alternative to the classical surgical treatment associated in patients of older age group with a severe course of the postoperative period and high lethality.

  15. 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.

  16. Aortic events in a nationwide Marfan syndrome cohort

    DEFF Research Database (Denmark)

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

    2017-01-01

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

  17. 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.)

  18. Right Aortic Arch and Kommerell’s Diverticulum Repaired without Reconstruction of Aberrant Left Subclavian Artery

    Directory of Open Access Journals (Sweden)

    Hiroshi Osawa

    2013-01-01

    Full Text Available Right aortic arch with Kommerell’s diverticulum is a very rare situation. Surgical treatment is recommended for symptomatic patients or asymptomatic patients with a large diverticulum. However planning the strategy of operation is difficult without a 3D imaging. We report a case of a 57-year-old man with right aortic arch, Kommerell’s diverticulum, and aberrant left subclavian artery. After a 3D-CT imaging, the patient underwent descending aortic replacement without reconstruction of aberrant left subclavian artery. After operation, there was no signs or symptoms of ischemia of the left arm. If the reconstruction of the aberrant subclavian artery was too difficult, closing its orifice is an acceptable decision. It has been found advantageous because of a decrease blood loss and a shorter cardiopulmonary bypass duration. If an ischemia of the arm is noticed, additional reconstruction will have to be considered. 3D-CT imaging was very useful to have a proper orientation and plan for the operative strategy.

  19. Repair of aortic arch aneurysm under cardiopulmonary bypass and deep hypothermia with low flow: A case report

    Directory of Open Access Journals (Sweden)

    Md. Rezwanul Hoque

    2016-07-01

    Full Text Available Aortic arch surgery is the challenging and most difficult surgery among the cardiovascular operations. Cerebral and spinal complications are the most feared and common complications of aortic arch surgery. With best available techniques for cerebral and spinal protection, anesthetic management and good post-operative care; aortic arch surgery is considerably safer nowadays and satisfactory results can be achieved in most patients. Also, selecting the sites for arterial cannulation to maintain whole body circulation, during isolation of the aortic arch to operate on it, need proper anatomical description of the extent of the aneurysm. This is also achievable by the availability of the imaging techniques like Computed Tomog­raphy (CT with or without contrast, CT Angiography (CTA and Magnetic Resonance Imaging (MRI. We are reporting a case of aneurysm of aortic arch in a young adult, who had undergone repair under cardiopulmonary bypass and deep hypothermia with low flow and had normal convalescence without any cerebral or spinal complications.

  20. Differences in aortic arch radius of curvature, neck size, and taper in patients with traumatic and aortic disease.

    Science.gov (United States)

    Alberta, Hillary B; Secor, Jessica L; Smits, Taylor C; Farber, Mark A; Jordan, William D; Matsumura, Jon S

    2013-09-01

    The purpose of this study was to determine the differences in aortic morphology that would potentially affect the management of thoracic endovascular aneurysm/aortic repair between trauma and aneurysm patients. This was a prospective analysis of the pretreatment digital imaging of 98 traumatic injury patients and 63 aneurysm patients enrolled in multicenter regulatory studies of the Conformable GORE TAG Thoracic Device (CTAG Device) (manufactured by W.L. Gore and Associates, Flagstaff, AZ). A standardized protocol was used to perform an independent assessment of the images and measurements of the radius of curvature and proximal and distal neck diameters. The radius of curvature was measured using axial images and the proximal and distal intimal neck diameter measurements were completed using the orthogonal "centerline" view. Taper was measured over the entire treated aorta and was calculated by subtracting the distal neck diameter measurement from the proximal neck diameter. The results were analyzed with independent t-tests. The trauma patients had a significantly smaller radius of curvature than aneurysm patients. There was a significant difference in the aortic neck size, with trauma patients having smaller proximal and distal intimal neck diameters. Taper was noted in trauma patients but not in aneurysm patients. The aortic anatomy varies between treated aortic pathologies. Aneurysm patients have a wider arch and larger aortas when compared with trauma patients. Aneurysm patients have less taper than trauma patients. Despite these differences, both of these cohorts of patients are treatable under the broader oversizing ranges of the CTAG Device. Copyright © 2013 Elsevier Inc. All rights reserved.

  1. Selective Heart, Brain and Body Perfusion in Open Aortic Arch Replacement.

    Science.gov (United States)

    Maier, Sven; Kari, Fabian; Rylski, Bartosz; Siepe, Matthias; Benk, Christoph; Beyersdorf, Friedhelm

    2016-09-01

    Open aortic arch replacement is a complex and challenging procedure, especially in post dissection aneurysms and in redo procedures after previous surgery of the ascending aorta or aortic root. We report our experience with the simultaneous selective perfusion of heart, brain, and remaining body to ensure optimal perfusion and to minimize perfusion-related risks during these procedures. We used a specially configured heart-lung machine with a centrifugal pump as arterial pump and an additional roller pump for the selective cerebral perfusion. Initial arterial cannulation is achieved via femoral artery or right axillary artery. After lower body circulatory arrest and selective antegrade cerebral perfusion for the distal arch anastomosis, we started selective lower body perfusion simultaneously to the selective antegrade cerebral perfusion and heart perfusion. Eighteen patients were successfully treated with this perfusion strategy from October 2012 to November 2015. No complications related to the heart-lung machine and the cannulation occurred during the procedures. Mean cardiopulmonary bypass time was 239 ± 33 minutes, the simultaneous selective perfusion of brain, heart, and remaining body lasted 55 ± 23 minutes. One patient suffered temporary neurological deficit that resolved completely during intensive care unit stay. No patient experienced a permanent neurological deficit or end-organ dysfunction. These high-risk procedures require a concept with a special setup of the heart-lung machine. Our perfusion strategy for aortic arch replacement ensures a selective perfusion of heart, brain, and lower body during this complex procedure and we observed excellent outcomes in this small series. This perfusion strategy is also applicable for redo procedures.

  2. Type A interrupted aortic arch accompanied by intracranial aneurysms causing subarachnoid hemorrhage in an adult man.

    Science.gov (United States)

    Eren, Suat; Kantarci, Mecit; Pirimoglu, Berhan; Cakir, Murteza; Ogul, Hayri

    2014-01-01

    Interrupted aortic arch anomaly (IAA) characterized by complete luminal dissociation between the ascending and descending aorta and accounting for less than 1% of all cases of congenital heart disease. IAA is a rare condition in infants that occurs approximately three times per million births. It is usually diagnosed and repaired during the neonatal period and is extremely rare in adults. We present the case of an adult man who was diagnosed with IAA accompanied by intracranial aneurysms causing subarachnoid hemorrhage and demonstrate the imaging findings with 256-slice computed tomography angiography and digital subtraction angiography. Copyright © 2014. Published by Elsevier Inc.

  3. Aortic aneurysm endovascular treatment with the parallel graft technique from the aortic arch to the iliac axis.

    Science.gov (United States)

    Fadda, Gian F; Marino, Mario; Kasemi, Holta; DI Angelo, Costantino L; Dionisi, Carlo P; Cammalleri, Valeria; Setacci, Carlo

    2017-05-26

    The chimney technique has been developed for the treatment of complex aortic aneurysms. We analyzed the midterm to long-term outcomes of this approach from a single- centre experience. From October 2008 to July 2016, 58 patients underwent endovascular aortic aneurysm repair using the chimney technique. Indications for treatment were thoracic aortic aneurysm (TAA) (n = 11), thoracoabdominal aortic aneurysm (TAAA) (n = 2), pararenal aortic aneurysm (PAAA) (n= 15), aortoiliac/isolated hypogastric artery aneurysm (n = 25), type I endoleak after previous TEVAR/EVAR (n=4), proximal pseudoaneurysm after AAA open repair (n = 1). Elective (82.8%) and emergent (17.2%) procedures were included. The immediate technical success was 100%. Single, double and triple chimneys were performed in 46, 10 and 2 patients, respectively. Overall, 61 target vessels (3 left common carotid arteries, 8 left subclavian arteries, 3 celiac trunks, 3 superior mesenteric arteries, 19 renal arteries and 25 hypogastric arteries) were involved. Post-operative mortality was 0. No neurologic complications were registered. Primary patency rate of the chimney stent/stent graft was 98.3%. Low flow type I endoleak was observed in 4 patients (6.9%). Post-operative chimney graft re-intervention rate was 1.7%. The median follow up was 32±20 months (range 3- 96 months). Overall estimated survival at 12, 50 and 80 months was 100%, 89% and 44%, respectively. Estimated freedom from endoleak at 1, 12, 24 and 36 months was 96.5%, 95%, 95% and 93%, respectively. One HA stent graft occluded at the 3rd month of follow up. No reintervention was performed. Our experience with the chimney technique for aortic aneurysms from the aortic arch to the iliac axis shows promising and durable mid- and long term results. Endograft oversizing, associated with the chimney graft diameter and length choice remain fundamental to reduce the risk of the most frequent procedure complications: type I endoleak and CG occlusion.The wider

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

    Science.gov (United States)

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

    2016-08-01

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

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

    OpenAIRE

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

    2012-01-01

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

  6. 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

  7. A schematic diagram showing the various components of the embryonic aortic arch complex in the retroesophageal right subclavian artery.

    Science.gov (United States)

    Kawai, Katsushi; Honma, Satoru; Kumagai, Yoshihiro; Koba, Yoshikazu; Koizumi, Masahiro

    2011-09-01

    A retroesophageal right subclavian artery, arising from the arch of the aorta as the terminal branch and passing dorsal to the esophagus, was found in five (1.2%) of 428 bodies donated for student dissection at Kumamoto University between 1993 and 2008. The presence of a retroesophageal right subclavian artery has been generally explained to be caused by the persistence of the normally eliminated part of the right dorsal aorta caudal to the seventh intersegmental artery and the disappearance of the normally patent right fourth aortic arch and the part of the right dorsal aorta cranial to the seventh intersegmental artery during the developmental process. However, the parts which remain or disappear are different in each case. With the aim of determining the portions eliminated or persisting and thereby gaining an understanding of the developmental process of the retroesophageal right subclavian artery in each instance, we made schematic diagrams showing the various components of the embryonic aortic arch complex as the prototype just before the anomaly occurred. Based on these diagrams, we conclude that immediately preceding the disappearance of the distal part of the right dorsal aorta and the dorsal part of the right sixth aortic arch, the third intersegmental artery was situated opposite to the fourth aortic arch and the seventh intersegmental artery was situated cranial to the point of junction of the right and left dorsal aortae.

  8. 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. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

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

    Science.gov (United States)

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

    2002-07-01

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

  10. [Hybrid treatment of patients with aneurysms and dissections of the aortic arch and descending portion of the thoracic aorta].

    Science.gov (United States)

    Shlomin, V V; Gordeev, M L; Zverev, D A; Shloĭdo, E A; Uspenskiĭ, V E; Zvereva, E D; Bondarenko, P B; Puzdriak, P D

    The authors share herein their experience with hybrid surgical treatment of 21 patients presenting with lesions of the aortic arch and descending thoracic aorta. Aortic pathology included dissection of the thoracoabdominal aorta (n=15), a sacciform aneurysm of the aortic arch (n=5), and a spindle-shaped aneurysm of the distal portions of the aortic arch (n=1). The first stage consisted of the following operations: transposition of the left subclavian artery into the left common carotid artery (n=9; 42.8%), partial debranching (n=11; 52.5%), and total debranching (n=1; 4.7%). The second stage consisted in implantation of a stent graft: to the thoracic aorta in 18 (85.8%) cases, and to the thoracic and abdominal portions of the aorta in 3 (14.2%) cases. The most significant complications of the immediate postoperative period included acute cerebral circulation impairment (n=1) and local dissection of the ascending aorta (n=1). Type I endoleaks were observed in 4 (19%) patients, type II endoleaks in 1 (4.7%), and type III endoleaks in 1 (4.7%). The mean duration of the follow up after discharge from hospital amounted to 11.6±7.9 months. In 4 patients after 6 months the findings of the control MSCT angiography showed no significant changes of the endoleaks. 1-year patency of the shunted branches of the aortic arch amounted to 95.2%. The cumulative survival rate amounted to 95.2%.

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

    Science.gov (United States)

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

    2007-02-01

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

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

    Science.gov (United States)

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

    2010-03-01

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

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

    Science.gov (United States)

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

    2012-12-01

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

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

    Science.gov (United States)

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

    2014-07-01

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

  15. 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 ...

  16. Preclinical 4D-flow magnetic resonance phase contrast imaging of the murine aortic arch.

    Directory of Open Access Journals (Sweden)

    Moritz Braig

    Full Text Available Cardiovascular diseases remain the number one death cause worldwide. Preclinical 4D flow phase contrast magnetic resonance imaging can provide substantial insights in the analysis of aortic pathophysiologies in various animal models. These insights may allow a better understanding of pathophysiologies, therapy monitoring, and can possibly be translated to humans. This study provides a framework to acquire the velocity field within the aortic arch. It analyses important flow values at different locations within the aortic arch. Imaging parameters with high temporal and spatial resolution are provided, that still allow combining this time-consuming method with other necessary imaging-protocols.A new setup was established where a prospectively gated 4D phase contrast sequence is combined with a highly sensitive cryogenic coil on a preclinical magnetic resonance scanner. The sequence was redesigned to maintain a close to steady state condition of the longitudinal magnetization and hence to overcome steady state artifacts. Imaging parameters were optimized to provide high spatial and temporal resolution. Pathline visualizations were generated from the acquired velocity data in order to display complex flow patterns.Our setup allows data acquisition with at least two times the rate than that of previous publications based on Cartesian encoding, at an improved image quality. The "steady state" sequence reduces observed artifacts and provides uniform image intensity over the heart cycle. This made possible quantification of blood speed and wall shear stress (WSS within the aorta and its branches. The highest velocities were observed in the ascending aorta with 137.5 ± 8 cm/s. Peak velocity values in the Brachiocephalic trunk were 57 ± 12 cm/s. Quantification showed that the peak flow occurs around 20 ms post R-wave in the ascending aorta. The highest mean axial wall shear stress was observed in the analysis plane between the left common carotid artery

  17. Persistent Aortic Arch Hypoplasia After Coarctation Treatment Is Associated With Late Systemic Hypertension.

    Science.gov (United States)

    Quennelle, Sophie; Powell, Andrew J; Geva, Tal; Prakash, Ashwin

    2015-06-25

    Mild transverse aortic arch (TAA) hypoplasia is common after coarctation treatment, but is considered benign in the absence of an arm-leg systolic blood pressure (SBP) difference. Hypertension (HTN) is a common long-term morbidity after successful coarctation treatment. We examined whether mild TAA hypoplasia after coarctation treatment is associated with late systemic HTN. We retrospectively reviewed 92 patients (median age, 19.9 years; range, 4.9 to 57.8; 60% male) 14.1±10.3 years after successful coarctation treatment (surgery in 63, stent in 16, and balloon dilation in 13), excluding those with resting right arm-leg blood pressure gradient >20 mm Hg, atypical coarctation, and major associated heart defects. Minimum body-surface area (BSA)-adjusted TAA cross-sectional area (CSA) was calculated from cardiac magnetic resonance (CMR) images. On follow-up, 38 of 92 (41%) patients had systemic HTN using standard criteria. Systemic HTN was independently associated with smaller TAA CSA/BSA (P=0.006; odds ratio [OR], 6.41 per 0.5 cm(2)/m(2) decrease), higher age at CMR (P=0.03; OR, 1.57 per 5-year increase), and in a subset (n=61), higher arm-leg SBP difference during exercise (P=0.05; OR, 1.03 per 1-mm-Hg increase). Lower ratio of TAA diameter/descending aorta diameter was associated with a larger increase in right arm SBP during peak exercise (P=0.006; r(2)=0.11). Persistent mild aortic arch hypoplasia, even in the absence of an arm-leg SBP difference at rest, is associated with late systemic HTN. Further studies should be undertaken to determine whether more-aggressive arch reconstruction at initial repair can reduce the incidence of systemic HTN. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

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

    Science.gov (United States)

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

    2014-05-01

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

  19. Surgical modification for preventing a gothic arch after aortic arch repair without the use of foreign material.

    Science.gov (United States)

    Seo, Dong-Man; Park, Jiyoung; Goo, Hyun Woo; Kim, Young Hwue; Ko, Jae-Kon; Jhang, Won Kyoung

    2015-04-01

    Systemic hypertension is the main late complication after arch reconstruction in patients with arch obstruction. Gothic arch geometry is suspected to be one of its possible causes. Accordingly, we evaluated here if a modified arch repair technique using an autologous pulmonary patch is effective in preventing gothic arch development. Fifty infants who underwent arch repair with either a modified (n = 17) or conventional (n = 33) technique between January 2006 and August 2012 by a single surgeon were retrospectively reviewed. Arch geometry was compared using three categories (gothic, crenel or roman), classified by the height/width (H/W) ratio and the arch angle measured in computed tomography. No gothic arch geometry was observed in the modified group, whereas it was observed in 9 cases in the conventional group (P = 0.005). Moreover, reintervention for arch restenosis was performed only in the conventional group (n = 4; P = 0.29). No associated complications were observed, although the selective cerebral perfusion time was longer in the modified group than in the conventional group (28.5 ± 6.2 vs 17.1 ± 9.9 min; P gothic arch geometry, but also as equally safe in terms of early clinical outcomes as conventional arch reconstruction techniques. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

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

    Science.gov (United States)

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

    2016-05-01

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

  1. 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

  2. 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.)

  3. First and second branchial arch syndromes: multimodality approach

    International Nuclear Information System (INIS)

    Senggen, Elodie; Laswed, Tarek; Meuwly, Jean-Yves; Maestre, Leonor Alamo; Meuli, Reto; Gudinchet, Francois; Jaques, Bertrand

    2011-01-01

    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.)

  4. Rapid Two-stage Versus One-stage Surgical Repair of Interrupted Aortic Arch with Ventricular Septal Defect in Neonates

    Directory of Open Access Journals (Sweden)

    Meng-Lin Lee

    2008-11-01

    Conclusion: The outcome of rapid two-stage repair is comparable to that of one-stage repair. Rapid two-stage repair has the advantages of significantly shorter cardiopulmonary bypass duration and AXC time, and avoids deep hypothermic circulatory arrest. LVOTO remains an unresolved issue, and postoperative aortic arch restenosis can be dilated effectively by percutaneous balloon angioplasty.

  5. Thoracic endograft positioning and carotid-subclavian bypass grafting in a patient with a saccular aneurysm at the aortic arch.

    Science.gov (United States)

    Valerio, Nicolas; Bucci, Federico; Redler, Adriano; Salvati, Bruno; Ferdani, Michel

    2007-01-01

    A 75-year-old man was referred to our hospital because of sudden thoracic pain. A saccular aneurysm of the aortic arch extending on the anterior surface of the aortic arch was found on computed tomographic arteriography. The patient was hemodynamically stable and he was programmed for a staged surgical and endovascular approach (hybrid approach). As a first stage and in order to prevent major cardiac complications due to the overstenting of the left subclavian artery (LSA) with the occlusion of the aortocoronary bypass, the patient underwent a polytetrafluoroethylene bypass graft (GORE-TEX, W.L. Gore & Associates, Flagstaff, AZ) between the LSA and the left carotid artery. Intraoperative arteriography revealed a good patency of the left carotid-subclavian bypass and of the left internal mammary bypass on the left anterior descending artery. As a second stage the endovascular procedure was accomplished 5 days later in the operating room. A Gore TAG stent graft (W.L. Gore & Associates) was deployed in the aortic arch 20 mm proximally to the aneurysmatic segment covering the ostium of the LSA. The postoperative course was uneventful and the patient was discharged on the fifth postoperative day in good general conditions. Hybrid procedures for treatment of aneurysms of the aortic arch or of the descending thoracic aorta are a promising alternative to open surgery especially in high-risk patients, with lower early morbidity and mortality rates. Long-term effectiveness remains to be fully elucidated.

  6. Stent implantation to ductus arteriosus in a patient with interrupted aortic arch guided by CT image overlay.

    Science.gov (United States)

    Goreczny, Sebastian; Dryzek, Pawel; Moszura, Tomasz

    2017-08-01

    A 15-day-old premature patient with ventricular septal defect and interrupted aortic arch type B underwent "hybrid" initial treatment consisting of bilateral pulmonary artery banding followed by stenting of the ductus arteriosus. A pre-registered CT scan was re-purposed with a new three-dimensional image fusion software (VesselNavigator) to create a roadmap for stent delivery.

  7. Aortic root surgery in Marfan syndrome.

    Science.gov (United States)

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

    2008-03-01

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

  8. Mobile thromboses of the aortic arch without aortic debris. A transesophageal echocardiographic finding associated with unexplained arterial embolism. The Filiale Echocardiographie de la Société Française de Cardiologie.

    Science.gov (United States)

    Laperche, T; Laurian, C; Roudaut, R; Steg, P G

    1997-07-01

    Atherosclerotic lesions of the aortic arch are potential sources of arterial embolism. Mobile thrombi in the aortic arch in young patients without diffuse atherosclerosis have been reported recently, but such cases remain exceptional. We describe a series of young patients with unexplained arterial embolism in whom transesophageal echocardiography detected mobile aortic arch thromboses. Transesophageal echocardiography files collected between 1991 and 1995 in French academic cardiology centers were reviewed to identify patients who fulfilled the following criteria: (1) an arterial embolic event in the preceding weeks; (2) a mobile pedunculated aortic arch thrombosis, defined as an echogenic mass protruding into the lumen of the aorta and inserted on the aortic arch; and (3) absence of obvious diffuse aortic atherosclerosis or of aortic debris on transesophageal echocardiography. Twenty-three cases were identified from 27 855 examinations. Thromboses were located on the horizontal aorta (n = 4), near the ostium of the left subclavian artery (n = 5), or on the concavity of the posterior segment of the aortic arch (in the isthmus) (n = 14). The insertion site was a small atherosclerotic plaque in 21 patients. The remaining aortic wall always appeared normal or mildly atherosclerotic. The mean age of the patients was 45 +/- 8.4 years (range, 26 to 61 years). All patients were treated with intravenous heparin after the diagnosis of aortic arch thrombosis, and surgical removal of the thrombosis was performed in 10 patients in whom histological examination confirmed an atherosclerotic process at the site of insertion of the thrombosis. The prognosis was mainly influenced by embolic events. Thromboses of the aortic arch appear to be a variant form of aortic atherosclerotic disease associated with arterial embolism in young patients.

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

    Science.gov (United States)

    Miyahara, Shunsuke; Okita, Yutaka

    2016-09-01

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

  10. Internal right ventricular band for multiple ventricular septal defects in a neonate undergoing arterial switch and aortic arch repair.

    Science.gov (United States)

    Carroll, William W; Shirali, Girish S; Bradley, Scott M

    2011-01-01

    A neonate presented with d-transposition of the great arteries, aortic arch hypoplasia, aortic coarctation, and multiple ventricular septal defects. During the arterial switch procedure and the aortic arch repair, a fenestrated Gore-Tex disk (W.L. Gore & Assoc, Flagstaff, AZ) was sewn into the right ventricular outflow tract to restrict pulmonary blood flow. The internal right ventricular band successfully controlled the pulmonary blood flow, maintaining a systemic oxygen saturation of 88% to 92%, and allowing growth from 3.5 to 10.5 kg. At 8 months of age, the internal band in the patient was removed, and the ventricular septal defects were successfully closed. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  11. Branching patterns of the aortic arch in the Siberian roe deer (Capreolus pygargus Pallas, 1771).

    Science.gov (United States)

    Shin, Sangyun; Sim, Jeong-Ha; Kim, Jong-Teak; Oh, Hong-Shik; Tae, Hyun-Jin; Park, Byung-Yong; Kim, In-Shik; Ahn, Dongchoon

    2018-02-02

    This study examined the branching pattern of the aortic arch (AA) and its major branches in the Siberian roe deer (Capreolus pygargus Pallas, 1771) from South Korea. A total of eight of the nine expected types, based on the branching site and bilateral levels of the costocervical trunk (CCT) and subclavian artery (SB), were observed in the arterial silicone casts of 35 deer (16 males, 19 females). This deer has no typical type. The three most common types were present in 28.6, 25.7 and 20.0% of cases and resulted from different branching patterns of the left CCT and left SB. These results suggest that the Siberian roe deer in the Korean peninsula has various AA branching patterns, which differs from other ruminants.

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

    Science.gov (United States)

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

    2002-07-01

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

  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

    International Nuclear Information System (INIS)

    Bangard, Christopher; Franke, Mareike; Maintz, David; Chang, De-Hua; Pfister, Roman; Deppe, Antje-Christin; Matoussevitch, Vladimir

    2014-01-01

    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. Outflow tract septation and the aortic arch system in reptiles: lessons for understanding the mammalian heart

    Directory of Open Access Journals (Sweden)

    Robert E. Poelmann

    2017-05-01

    Full Text Available Abstract Background Cardiac outflow tract patterning and cell contribution are studied using an evo-devo approach to reveal insight into the development of aorto-pulmonary septation. Results We studied embryonic stages of reptile hearts (lizard, turtle and crocodile and compared these to avian and mammalian development. Immunohistochemistry allowed us to indicate where the essential cell components in the outflow tract and aortic sac were deployed, more specifically endocardial, neural crest and second heart field cells. The neural crest-derived aorto-pulmonary septum separates the pulmonary trunk from both aortae in reptiles, presenting with a left visceral and a right systemic aorta arising from the unseptated ventricle. Second heart field-derived cells function as flow dividers between both aortae and between the two pulmonary arteries. In birds, the left visceral aorta disappears early in development, while the right systemic aorta persists. This leads to a fusion of the aorto-pulmonary septum and the aortic flow divider (second heart field population forming an avian aorto-pulmonary septal complex. In mammals, there is also a second heart field-derived aortic flow divider, albeit at a more distal site, while the aorto-pulmonary septum separates the aortic trunk from the pulmonary trunk. As in birds there is fusion with second heart field-derived cells albeit from the pulmonary flow divider as the right 6th pharyngeal arch artery disappears, resulting in a mammalian aorto-pulmonary septal complex. In crocodiles, birds and mammals, the main septal and parietal endocardial cushions receive neural crest cells that are functional in fusion and myocardialization of the outflow tract septum. Longer-lasting septation in crocodiles demonstrates a heterochrony in development. In other reptiles with no indication of incursion of neural crest cells, there is either no myocardialized outflow tract septum (lizard or it is vestigial (turtle. Crocodiles

  16. [Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring].

    Science.gov (United States)

    Tamayo-Espinosa, Tania; Erdmenger-Orellana, Julio; Becerra-Becerra, Rosario; Balderrabano-Saucedo, Norma; Segura-Standford, Begoña

    The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly. Copyright © 2017 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

  17. Whole body perfusion for hybrid aortic arch repair: evolution of selective regional perfusion with a modified extracorporeal circuit.

    Science.gov (United States)

    Fernandes, Philip; Walsh, Graham; Walsh, Stephanie; O'Neil, Michael; Gelinas, Jill; Chu, Michael W A

    2017-04-01

    Patients undergoing hybrid aortic arch reconstruction require careful protection of vital organs. We believe that whole body perfusion with tailored dual circuitry may help to achieve optimal patient outcomes. Our circuit has evolved from a secondary circuit utilizing a cardioplegia delivery device for lower body perfusion to a dual-oxygenator circuit. This allows individually controlled regional perfusion with ease of switching from secondary to primary circuit for total body flow. The re-design allows for separate flow and temperature regulation with two oxygenators in parallel. All patients underwent a single-stage operation for simultaneous treatment of arch and descending aortic pathology via a sternotomy, using a hybrid frozen elephant trunk technique. We report six consecutive patients undergoing hybrid arch and frozen elephant trunk reconstruction using a dual-oxygenator circuit. Five patients underwent elective surgery and one was emergent. One patient had an acute dissection while three underwent concomitant procedures, including a Ross procedure and two valve-sparing root reconstructions. Three cases were redo sternotomies. The mean pump time was 358 ± 131 min, the aortic cross clamp time 243 ± 135 min, the cardioplegia volume of 33,208 ml ± 16,173, cerebral ischemia 0 min, lower body ischemia 76 ± 34 min and the average lower body perfusion time was 142 min. Two patients did not require any donor blood products. The median intensive care unit (ICU) and hospital lengths of stay (LOS) were two days and 10 days, respectively. The average peak serum lactate on CPB was 7.47 mmol/L and, at admission to the ICU, it was 3.37 mmol/L. Renal and respiratory failure developed in the salvage acute type A dissection patient. No other complications occurred in this series. Whole body perfusion as delivered through individually controlled dual-oxygenator circuitry allows maximum flexibility for hybrid aortic arch reconstruction. A modified circuit perfusion

  18. 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.

  19. 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.

  20. Low-flow antegrade cerebral perfusion attenuates early renal and intestinal injury during neonatal aortic arch reconstruction.

    Science.gov (United States)

    Algra, Selma O; Schouten, Antonius N J; van Oeveren, Wim; van der Tweel, Ingeborg; Schoof, Paul H; Jansen, Nicolaas J G; Haas, Felix

    2012-12-01

    Deep hypothermic circulatory arrest (DHCA) and antegrade cerebral perfusion (ACP) are 2 cardiopulmonary bypass strategies mainly used in aortic arch reconstructions. It has been suggested that during ACP, abdominal organs are better protected than during DHCA owing to partial perfusion via collaterals. We tested this hypothesis using intraoperative near-infrared spectroscopy (NIRS), lactate measurements, and biomarkers for early abdominal injury in neonates undergoing complex aortic arch repair. Neonates scheduled for aortic arch reconstruction via median sternotomy between 2009 and 2011 were randomized to either DHCA or ACP. During surgery, regional oxygen saturations of the abdomen were monitored using NIRS. Immediately aafter DHCA or ACP, lactate concentrations from the inferior vena cava were compared with those from the arterial cannula. Postoperatively, biomarkers for early abdominal organ injury were measured in urine. Twenty-five neonates were analyzed (DHCA, n = 12; ACP, n = 13). Procedures were performed at 18°C, and ACP flow was set at 35 to 50 mL · kg(-1) · min(-1). Median abdominal NIRS value during DHCA was 31% (IQR, 28%-41%) whereas during ACP it was 56% (IQR, 34%-64%; P intestinal damage (gluthatione s-transferase and intestinal fatty acid binding protein, respectively) were higher in the DHCA group than for the ACP group (P = .03, P = .04, respectively). These results substantiate earlier suggestions that ACP provides more abdominal organ protection than DHCA in neonates undergoing aortic arch reconstruction. Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  1. Modern temperature management in aortic arch surgery: the dilemma of moderate hypothermia.

    Science.gov (United States)

    Luehr, Maximilian; Bachet, Jean; Mohr, Friedrich-Wilhelm; Etz, Christian D

    2014-01-01

    Arch surgery is undoubtedly among the most technically and strategically challenging endeavours in aortic surgery, requiring thorough understanding not only of cardiovascular physiology, but also in particular, of neurophysiology (cerebral and spinal cord), and is still associated with significant mortality and morbidity. In the late 1980s, when deep hypothermic circulatory arrest (HCA) had gained widespread acceptance as the standard approach for arch surgery, antegrade selective cerebral perfusion (SCP), as an adjunct to deep HCA, began its triumphal march, offering excellent neuroprotection and improved overall outcome. This encouraged the use of antegrade SCP in combination with steadily increasing body core temperatures--a trend culminating in the progressive advocation of moderate-to-mild temperatures up to 35 °C, and even normothermia. The impetus for progressive temperature elevation was the limitation of adverse effects of profound hypothermia and the most welcome side effect of significantly shorter cooling and rewarming periods on cardiopulmonary bypass (CPB), and thereby, potentially, the alleviation of the systemic inflammatory response and, in particular, the risk of severe postoperative bleeding (and other organ dysfunctions). The safe limits of prolonged distal circulatory arrest, particularly with regard to the ischaemic tolerance of the viscera and the spinal cord, have not yet been clearly defined. Adverse outcomes due to inappropriate temperature management (core temperatures too high for the required duration of distal arrest) are probably highly underreported. Complications historically associated with hypothermia, namely excessive bleeding, are possibly overestimated. Trading effective neuroprotection and excellent outcomes for the risk of prolonged 'warm' distal ischaemia might constitute a significant step back, jeopardizing visceral and, in particular, spinal cord integrity, with unpredictable consequences for long-term outcome and

  2. Long-term Voice Outcomes of Early Thyroplasty for Unilateral Vocal Fold Paralysis Following Aortic Arch Surgery.

    Science.gov (United States)

    Kwak, Paul E; Tritter, Andrew G; Donovan, Donald T; Ongkasuwan, Julina

    2016-07-01

    To describe this institution's experience with and the long-term outcomes of early type 1 thyroplasty for unilateral vocal fold paralysis (UVFP) following surgery on the aortic arch. Retrospective chart review with telephone questionnaire. Academic tertiary care center. Three hundred forty-eight patients with UVFP following surgery on the aortic arch since 1999 were identified; 40 were available for follow-up. The number of revision procedures following initial thyroplasty was ascertained, and the Voice Handicap Index (VHI) was administered by telephone. The hypothesis that early thyroplasty produced voice outcomes and revision rates comparable to injection laryngoplasty was established prior to the initiation of data collection. Six out of the 40 patients (15%) required revision thyroplasty following their initial procedure. Mean VHI of all patients was 36.0 (SD, 27.2). Mean VHI was significantly different in the 18 to 39 age group (13.1) when compared to the 40 to 59 (51.8) and 60+ (37.7) age groups (P = .013). Mean follow-up since initial thyroplasty was 46.5 months (SD, 42.2). In the setting of aortic arch surgery with injury to the recurrent laryngeal nerve, early thyroplasty produces voice outcomes comparable to those achieved in the literature with repeated injection and delayed thyroplasty and can be considered in select populations. © The Author(s) 2016.

  3. 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...

  4. A longitudinal study of dental arch morphology in children with the syndrome of Crouzon or Apert

    NARCIS (Netherlands)

    Reitsma, J.H.; Elmi, P.; Ongkosuwito, E.M.; Buschang, P.H.; Prahl-Andersen, B.

    2013-01-01

    The aim of this study was to compare changes in dental arch morphology between patients with Crouzon syndrome or Apert syndrome and controls. Children between 4 and 14 yr of age with Crouzon syndrome (n = 40) or Apert syndrome (n = 28) were compared with non-syndromic controls (n = 457) in terms of

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

    Science.gov (United States)

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

    2015-01-27

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

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

    Science.gov (United States)

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

    2011-08-01

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

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

    Directory of Open Access Journals (Sweden)

    Maria C. Guido

    2018-01-01

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

  8. Persistent right aortic arch and aberrant left subclavian artery in a white Bengal tiger (Panthera tigris).

    Science.gov (United States)

    Ketz, C J; Radlinsky, M; Armbrust, L; Carpenter, J W; Isaza, R

    2001-06-01

    A 3-mo-old male white Bengal tiger (Panthera tigris) presented with the chief complaint of regurgitation of solid food since weaning at 2 mo of age. Compared with its littermates, the tiger was in poor body condition and weighed only 10.3 kg when its littermates were estimated at 20-25 kg. Thoracic radiographs showed a megaesophagus cranial to the heart base. A contrast esophagram more clearly outlined the megaesophagus, and fluoroscopy demonstrated normal motility of the caudal esophagus. Endoscopic examination revealed a structure coursing dorsally from right to left over the esophagus and a constrictive band on the left of the esophagus at the heart base. Nonselective angiography confirmed the presence of a persistent right aortic arch, as well as an aberrant left subclavian artery. A left fourth intercostal thoracotomy was performed, and the ligamentum arteriosum was double ligated and divided. The left subclavian artery did not cause significant compromise of the esophagus and was not manipulated at surgery. The tiger recovered well from anesthesia and surgery. Solid food was slowly introduced over a 2-mo period without any regurgitation. The cub gained weight rapidly after surgery.

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

    Science.gov (United States)

    Zamani, Jalal; Aghasadeghi, Kamran; Zarrabi, Khalil; Abdi Ardekani, Alireza; Zolghadrasli, Abdolali

    2016-01-01

    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.

  10. 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.

  11. Aortic events in a nationwide Marfan syndrome cohort.

    Science.gov (United States)

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

    2017-02-01

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

  12. The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair.

    Science.gov (United States)

    Plymale, Jennifer M; Frommelt, Peter C; Nugent, Melodee; Simpson, Pippa; Tweddell, James S; Shillingford, Amanda J

    2017-08-01

    In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤-2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤-3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤-2.5 increased the odds of RI (OR 3.8; CI 1.3-11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from -2.34 to 0.04 (p indices. In this complex population, 1 year survival is high, but

  13. Open repair management of a patient with aortic arch saccular aneurysm, penetrating atherosclerotic ulcer, one vessel coronary artery disease and an isolated dissection of the abdominal aorta.

    Science.gov (United States)

    Romolo, Harvey; Wartono, Dicky A; Suyuti, Sugisman; Herlambang, Bagus; Caesario, Michael; Sunu, Ismoyo

    2017-01-01

    Isolated saccular compared to fusiform aneurysm is considered to be a rare entity with challenges of its own. A 62-year-old female was diagnosed with a case of saccular aneurysm and penetrating atherosclerotic ulcer of the aortic arch. Additionally, she also had one vessel coronary artery disease and type B abdominal aortic dissection. She was then managed with open aortic arch repair and coronary artery bypass grafting. If required, elective endovascular repair will be done for the abdominal aorta on a later date.

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

    Science.gov (United States)

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

    2004-02-01

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

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

    OpenAIRE

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

    2003-01-01

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

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

    NARCIS (Netherlands)

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

    2004-01-01

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

  17. Early- and Mid-term Results of the Chimney Technique in the Repair of Aortic Arch Pathologies

    Energy Technology Data Exchange (ETDEWEB)

    Zou, Junjie; Jiao, Yuanyong, E-mail: wishlucky@163.com, E-mail: johemail@163.com; Zhang, Xiwei; Jiang, Jun; Yang, Hongyu; Ma, Hao [First Affiliated Hospital of Nanjing Medical University, Division of Vascular Surgery, Department of General Surgery (China)

    2016-11-15

    PurposeTo examine the safety, feasibility, and mid-term efficacy of the chimney technique for aortic arch pathologies.MethodsFrom February 2011 to December 2014, a total of 35 patients (30 men; mean age 54.3 ± 14.1 years) with aortic arch pathologies underwent thoracic endovascular aortic repair combined with chimney stents. The indication was a proximal landing zone <1.5 cm. Follow-up was performed at 3, 6, and 12 months and then yearly thereafter.ResultsA total of 36 chimney stents were deployed (innominate artery, n = 1; left common carotid artery, n = 9; right subclavian artery, n = 1; left subclavian artery, n = 25). The technical success rate was 94.3 % (33/35). Immediate type Ia endoleaks (ELIa) were observed in two patients (8.6 %, 2/35). Twenty-five patients were successfully followed-up for a median period of 29.3 months (range, 6–48 months). One patient died due to aortic dissection aneurysm rupture at 36 months (mortality rate of 4 %, 1/25). Three late ELIa were observed and no reinterventions were performed. The overall incidence of ELIa was 20 % (5/25). During follow-up, the patency rate for chimney stents was 92 % (23/25).ConclusionOur limited experience demonstrates that the chimney technique is a viable and relatively safe treatment for patients with challenging thoracic aortic pathologies at least in the mid-term follow-up period.

  18. Thoracoscopy with Concurrent Esophagoscopy for Persistent Right Aortic Arch in 9 Dogs.

    Science.gov (United States)

    Townsend, Sarah; Oblak, Michelle L; Singh, Ameet; Steffey, Michele A; Runge, Jeffrey J

    2016-11-01

    To report the diagnosis, treatment, and short-term outcome in dogs with suspected persistent right aortic arch (PRAA) undergoing thoracoscopy with concurrent esophagoscopy. Multi-institutional retrospective case series. Dogs with suspected PRAA (n=9). Medical records were reviewed from 2012 to 2016. Dogs undergoing thoracoscopy for PRAA at 3 referral hospitals were included. Signalment, clinical signs, diagnostic imaging, anesthesia protocol (including the use of one-lung ventilation), surgical approach, complications, and short-term outcome were recorded. Dogs underwent a left-sided intercostal thoracoscopic approach with concurrent intraoperative esophagoscopy. The ligamentum arteriosum (LA) and constricting fibers were divided using a vessel-sealing device using a 3 or 4 port thoracoscopy technique. Visualization and dissection of the LA was aided by transesophageal illumination by esophagoscopy. Thoracoscopy confirmed PRAA in 9 dogs, with an aberrant left subclavian artery (LS) identified in 5 dogs. Major complications occurred in 2 dogs: postoperative hemorrhage from the LS and esophageal perforation, which resulted in euthanasia. Median follow-up was 250 days (range, 56-1,595). Regurgitation resolved in 4 of 8 surviving dogs. One dog had recurrence of regurgitation 1,450 days postoperatively, esophageal compression by the LS was identified, and regurgitation resolved following LS transection. Esophagoscopy aided identification and dissection of the LA in all cases. Due to the potential for the LS to cause clinical esophageal constriction postoperatively, a recommendation for LS transection may be warranted. Vascular clips can also be considered as an alternative for vessel ligation to avoid complications associated with vessel-sealing device use. © Copyright 2016 by The American College of Veterinary Surgeons.

  19. Aortic arch origin of the left vertebral artery: An Anatomical and Radiological Study with Significance for Avoiding Complications with Anterior Approaches to the Cervical Spine.

    Science.gov (United States)

    Tardieu, Gabrielle G; Edwards, Bryan; Alonso, Fernando; Watanabe, Koichi; Saga, Tsuyoshi; Nakamura, Moriyoshi; Motomura, Mayuko; Sampath, Raghuram; Iwanaga, Joe; Goren, Oded; Monteith, Stephen; Oskouian, Rod J; Loukas, Marios; Tubbs, R Shane

    2017-09-01

    Complications from anterior approaches to the cervical spine are uncommon with normal anatomy. However, variant anatomy might predispose one to an increased incidence of injury during such procedures. We hypothesized that left vertebral arteries that arise from the aortic arch instead of the subclavian artery might take a more medial path in their ascent making them more susceptible to iatrogenic injury. Fifty human adult cadavers were examined for left vertebral arteries having an aortic arch origin and these were dissected along their entire cervical course. Additionally, two radiological databases of CTA and arteriography procedures were retrospectively examined for cases of aberrant left vertebral artery origin from the aortic arch over a two-year period. Two cadaveric specimens (4%) were found to have a left vertebral artery arising from the aortic arch. The retrospective radiological database analysis identified 13 cases (0.87%) of left vertebral artery origin from the aortic arch. Of all cases, vertebral arteries that arose from the aortic arch were much more likely to not only have a more medial course (especially their preforaminal segment) over the cervical vertebral bodies but also to enter a transverse foramen that was more cranially located than the normal C6 entrance of the vertebral artery. Spine surgeons who approach the anterior cervical spine should be aware that an aortic origin of the left vertebral artery is likely to be closer to the midline and less protected above the C6 vertebral level. Clin. Anat. 30:811-816, 2017. © 2017Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

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

    Science.gov (United States)

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

    2017-01-01

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

  1. Spontaneous dissection of the arterial duct during continuous infusion of prostaglandin E1 in a neonate with aortic arch interruption.

    Science.gov (United States)

    Inoue, Nao; Yasukochi, Satoshi; Takigiku, Kiyohiro; Matsui, Hikoro; Takei, Kohta; Nakano, Yusuke; Otagiri, Tessyu; Hashida, Yuichiro; Ogiso, Yoshifumi; Maekawa, Yoshiyuki; Umezu, Kentaro; Sakamoto, Takahiko; Harada, Yorikazu

    2013-12-01

    We report a 3-day-old boy with double outlet of the right ventricle and interruption of the aortic arch who developed spontaneous dissection of the arterial duct (DA) despite use of continuous infusion of lipo-prostaglandin E1 (PGE1). Transthoracic echocardiography demonstrated the spontaneous dissecting aneurysm of DA, which was confirmed by histology at the modified Norwood procedure done at age of 18 days. This is the first report of spontaneous dissection of DA in a neonate receiving PGE1, suggesting a new closing mechanism of DA.

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

    Science.gov (United States)

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

    2015-06-09

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

  3. 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.

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

    Science.gov (United States)

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

    2015-09-29

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

  5. 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.

  6. Bentall operation, total aortic replacement and mitral valve replacement for a young adult with Marfan syndrome: a case of three-staged operation.

    Science.gov (United States)

    Inui, K; Shimazaki, Y; Watanabe, T; Kuraoka, S; Minowa, T; Miura, M; Oshikiri, S; Toyama, H

    1998-08-01

    In Marfan syndrome, the most common cardiovascular abnormalities are dilatation of the aorta and aortic valve regurgitation in adult patients. Mitral valve dysfunction is the most common cause of morbidity and mortality in infants and children with Marfan syndrome, and is not frequently operated on in adult Marfan patients who undergo surgery for diseases of the aortic root and total aorta. This report describes a successfully three-staged operation for a 24 year-old man with Marfan syndrome who underwent an emergent Bentall operation and aortic arch replacement, total aortic replacement and mitral valve replacement over 2 years. Mitral valve regurgitation was mild but increased after the second operation. The graft was tightly adhesive and invasive to the sternum. Endoscopic view was helpful to avoid graft damage at resternotomy. The postoperative course was uneventful in each operation. Microscopic examination of the mitral valve leaflets showed abnormal increase of mucopolysaccharides, and disruption and fragmentation of elastic fibers.

  7. 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.)

  8. Successful Embolization Therapy through Reentry Tear in the Right Subclavian Artery for Treating Patent False Lumen in the Aortic Arch Formed after Type A Dissection Repair

    Science.gov (United States)

    Ishii, Hirohito; Nakamura, Kunihide; Nakamura, Eisaku; Furukawa, Koji; Ochiai, Kouichiro

    2017-01-01

    A 73-year-old woman had undergone hemiarch replacement with primary entry resection for treating acute type A dissection 6 years ago. Postoperative computed tomography (CT) showed a patent false lumen (FL) in the aortic arch and a reentry tear in the right subclavian artery. The remaining aortic arch enlarged, which resulted in formation of a 55-mm-diameter aneurysm. We performed reentry occlusion using embolization with glue and coil. The patient’s clinical course after the procedure was uneventful, and subsequent CT showed that FL was thrombosed and had decreased in size. PMID:29147154

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

    OpenAIRE

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

    2012-01-01

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

  10. Utility of fetal cardiac magnetic resonance imaging to assess fetuses with right aortic arch and right ductus arteriosus.

    Science.gov (United States)

    Dong, Su-Zhen; Zhu, Ming

    2018-06-01

    To evaluate the utility of fetal cardiac magnetic resonance imaging (MRI) to diagnose right aortic arch (RAA) with right ductus arteriosus. This retrospective study included six fetuses with right aortic arch and right ductus arteriosus. The six fetal cases were examined using a 1.5-T magnetic resonance unit. The steady-state free precession (SSFP) and single-shot turbo spin echo (SSTSE) sequences were used to evaluate the fetal heart and airway. The gestational age of the six fetuses ranged from 22 to 35 weeks (mean, 26.5 weeks). The age of the pregnant women ranged from 23 to 40 years (mean 31 years). Fetal cardiac MRI diagnosed the six fetal cases with RAA with right ductus arteriosus correctly. Among the six fetuses, four were associated with other congenital heart defects. In three of six cases, the diagnoses established using prenatal echocardiography (echo) was correct when compared with postnatal diagnosis. Fetal cardiac MRI is a useful complementary tool to assess fetuses with RAA and right ductus arteriosus.

  11. Aortic valve-sparing surgery in Marfan syndrome.

    Science.gov (United States)

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

    2013-08-01

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

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

    Science.gov (United States)

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

    2003-01-01

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

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

    Science.gov (United States)

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

    2003-01-01

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

  14. Reoperation for right ventricular outflow tract obstruction after arterial switch operation for transposition of the great arteries and aortic arch obstruction

    NARCIS (Netherlands)

    Bokenkamp, Regina; Aguilar, Elizabeth; van der Palen, Roel L. F.; Sojak, Vladimir; Bruggemans, Eline F.; Hruda, Jaroslav; Kuipers, Irene M.; Hazekamp, Mark G.

    2016-01-01

    Right ventricular outflow tract obstruction (RVOTO) is one of the reasons for late reinterventions after repair of transposition of the great arteries (TGA) with aortic arch obstruction (AAO). The aim of the present study was to identify predictors of reoperation for RVOTO in patients who underwent

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

    Science.gov (United States)

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

    2007-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Marcelo Cury

    2013-01-01

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

  17. Segmentation and quantification of the aortic arch using joint 3D model-based segmentation and elastic image registration.

    Science.gov (United States)

    Biesdorf, Andreas; Rohr, Karl; Feng, Duan; von Tengg-Kobligk, Hendrik; Rengier, Fabian; Böckler, Dittmar; Kauczor, Hans-Ulrich; Wörz, Stefan

    2012-08-01

    Accurate quantification of the morphology of vessels is important for diagnosis and treatment of cardiovascular diseases. We introduce a new joint segmentation and registration approach for the quantification of the aortic arch morphology that combines 3D model-based segmentation with elastic image registration. With this combination, the approach benefits from the robustness of model-based segmentation and the accuracy of elastic registration. The approach can cope with a large spectrum of vessel shapes and particularly with pathological shapes that deviate significantly from the underlying model used for segmentation. The performance of the approach has been evaluated on the basis of 3D synthetic images, 3D phantom data, and clinical 3D CTA images including pathologies. We also performed a quantitative comparison with previous approaches. Copyright © 2012 Elsevier B.V. All rights reserved.

  18. ATTEMPTED SURGICAL CORRECTION OF A PERSISTENT RIGHT FOURTH AORTIC ARCH IN A JUVENILE ROTHSCHILD'S GIRAFFE (GIRAFFA CAMELOPARDALIS ROTHSCHILDI).

    Science.gov (United States)

    Waugh, Lynnette; D'Agostino, Jennifer; Cole, Gretchen A; Hahn, Alicia; Rochat, Mark; Sula, Mee Ja M

    2017-06-01

    A 5-mo-old female Rothschild's giraffe ( Giraffa camelopardalis rothschildi) presented for regurgitation. Esophagoscopy at 24 wk of age revealed a markedly dilated cranial esophagus with a tight stricture at the level of the heart base consistent with a vascular ring anomaly. Surgical exploration confirmed persistent right fourth aortic arch with ductus originating from left subclavian artery at its junction with the aorta and left subclavian artery. The patent ductus arteriosus was surgically ligated. The procedure was complicated by limited surgical access and vascular friability resulting in uncontrollable hemorrhage, and the animal was euthanatized. The animal's large size and unique shape precluded preoperative examination by computed tomography. Surgical accessibility was poor because cranial retraction of the thoracic limb was limited. Histology revealed focal degeneration of the aorta and subclavian artery and muscular degeneration of the esophagus. Degeneration was attributed to local hypoxia from compression by the vascular structure as the animal grew.

  19. Association of calcified carotid atheromas visualized on panoramic images and aortic arch calcifications seen on chest radiographs of postmenopausal women.

    Science.gov (United States)

    Friedlander, Arthur H; El-Saden, Suzie M; Aghazadehsanai, Nona; Chang, Tina I; Harada, Nancy D; Garrett, Neal R

    2014-04-01

    Occult atherosclerotic disease is the leading cause of death among older women. The authors hypothesized that women with calcified carotid artery plaque (CCAP) visualized on panoramic images were more likely to have aortic arch calcifications (AAC) that were visible on chest radiographs (CRs), a risk indicator of experiencing cardiovascular events, than would matched cohorts who did not have atheromas. The authors obtained the CRs of 36 female veterans (≥ 50 years) who had CCAP and atherogenically risk-matched them to those of 36 women without CCAP. A radiologist evaluated the CRs for AAC. Other study variables included age, ethnicity, body mass index and presence or absence of hypertension, diabetes and dyslipidemia. The authors computed descriptive and bivariate statistics. Women 60 years or older who had evidence of CCAP on their panoramic radiographs were significantly (P = .022; 95 percent confidence interval, 1.298-26.223) more likely to have evidence of AAC on their CRs than were similarly aged women who did not have evidence of CCAP. This association was not evident in women younger than 60 years. Among women who were both younger and older than 60 years, there was no evident association between the presence of CCAP and the severity (on a four point scale [0-3]) of AAC calcification. Prevalence of carotid plaque on panoramic images of women 60 years or older is significantly associated with presence of aortic arch calcifications on CRs. Panoramic images of women 60 years or older must be evaluated for CCAP, given their association with AAC. Patients with atheromas should be referred to their physicians for further evaluation given the systemic implications.

  20. The knee alignment and the foot arch in patients with Turner syndrome.

    Science.gov (United States)

    Trzcińska, Dorota; Olszewska, Elżbieta; Wiśniewski, Andrzej; Milde, Katarzyna; Madej, Marcin

    2011-01-01

    It is established that patients with Turner syndrome (TS) have numerous defects of the skeletal system, including in the lower extremities structure. However, there are not enough studies in the literature about knee alignment and foot arches in girls and women with TS. Assessment of knees and feet in girls and women with TS. Sixty-two girls and women with TS were examined. The mean chronological age of the patients was 15.61±5.27 years, ranging from 6.36 to 27.04-years-old. All patients underwent physical examinations of their knees and feet. Additionally, almost 70% (n=42) underwent plantographic examinations and measurement of their heel angle and Clarke's angle. The formation of the foot in patients with TS was related to the reference values developed on the basis of studies of healthy girls (n=92). In women whose knee conditions were assessed only on the basis of a physical examination, a drawer test was performed each time to assess the stability of the knee ligament systems. In over 60% of patients with TS, incorrect knee alignment, primarily in the form of genu valgum (knock-knee), was diagnosed. At the same time, 60% of the patients tested only in physical examinations were diagnosed with knee ligament instabilities (positive drawer test). Physical examinations showing foot arch malformation - in equal proportion splay-foot and low-arched - were found in almost two-thirds of women with TS. However, on the basis of plantography, abnormalities in the longitudinal arch of the foot were diagnosed in 60% of patients with TS, and abnormalities in the transverse arch of the foot were diagnosed in 80% of patients with TS. In patients with TS, the occurrence of the following is characteristic: 1. Genu valgum of knees and heels; 2. Frequent cases of abnormally formed longitudinal and transverse arches of the foot, with the longitudinal arch of the foot usually being reduced, while the transverse arch is excessively elevated; 3. Asymmetry involving a frequent

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Science.gov (United States)

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

    2012-02-01

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

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

    Science.gov (United States)

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

    2012-06-01

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

  4. 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.)

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

    International Nuclear Information System (INIS)

    Frydrychowicz, Alex; Berger, Alexander; Russe, Maximilian F.; Bock, Jelena; Munoz del Rio, Alejandro; Harloff, Andreas; Markl, Michael

    2012-01-01

    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.)

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

    Science.gov (United States)

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

    2015-01-01

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

  7. 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. Copyright © 2016 Elsevier Inc. All rights reserved.

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

    OpenAIRE

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

    2011-01-01

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

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

    African Journals Online (AJOL)

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

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

    Science.gov (United States)

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

    2014-06-01

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

  11. Acute Aortic Dissection in Pregnancy with the Marfan Syndrome

    OpenAIRE

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

    2014-01-01

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

  12. Frozen elephant trunk reconstruction for right-sided aortic arch with aberrant left subclavian artery and aneurysm of the descending aorta: a case report.

    Science.gov (United States)

    Kokotsakis, John; Jarral, Omar A; Harling, Leanne; Tsipas, Panteleimon; Athanasiou, Thanos

    2016-05-05

    A 59-year old man being investigated for back pain was found to have aneurysmal dilatation of a right-sided aortic arch and descending thoracic aorta together with an aberrant left subclavian artery. He underwent repair of this utilising the frozen elephant trunk technique, which dealt with all three pathologies in one-stage. He made an unremarkable recovery and was discharged home on the 8th post-operative day. This case report further demonstrates the flexibility and safety of the frozen elephant trunk in dealing with complex aortic pathology as a single-stage procedure.

  13. Expanding arch aneurysm causing a "kink" in a Bentall graft and heart failure.

    Science.gov (United States)

    Al-Mohaissen, Maha A; Skarsgard, Peter; Khoo, Clarence; Virani, Sean A; Munt, Brad; Leipsic, Jonathon; Ignaszewski, Andrew

    2012-07-01

    Marfan syndrome is associated with a high incidence of aortic root aneurysm and life-threatening aortic dissection. With the successful use of surgical aortic root replacement, dissection-related mortality has been significantly reduced. We present the case of a patient with Marfan syndrome who presented with heart failure secondary to an unusual graft-related complication 14 years after a Bentall procedure. Investigations revealed a supra-aortic stenosis resulting from a kink in the Bentall graft caused by pressure from an expanding aortic arch aneurysm. The patient underwent surgery with improvement in his ejection fraction and heart failure symptoms.

  14. Arterial hypertension in patients with repaired coarctation and hypoplastic aortic arch

    Directory of Open Access Journals (Sweden)

    И. А. Сойнов

    2015-10-01

    Full Text Available At present, surgical techniques for treatment of CoA are well established and the mortality is one of the lowest amongst all CHD patients. Nevertheless, the incidence of arterial hypertension (AH, cerebrovascular disease and heart disease is still high even in those patients who have no signs of re-coarctation and deformity of aorta. According to several modern studies, these problems are thought to be due to microstructural changes of the aortic wall (disorders of the collagen-elastic carcass, malfunctions of the aortic baroreceptors and unnatural geometry of the aorta after surgery. This study was designed to analyze the possible causes of residual AH after surgical treatment of coarctation and hypoplasia of the aorta. A high incidence of residual AH is still a topical issue for this group of patients and requires further research of pathogenesis and optimal therapies of this disorder.

  15. A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right-sided aortic arch

    Directory of Open Access Journals (Sweden)

    Trilok Chand

    2017-01-01

    Full Text Available The absence of one of the pulmonary artery with associated hypoplasia of lung and great vessel abnormality is a rare finding. The incidence of this rare congenital abnormality is around 1 in 200,000 live birth. The absence of the left side pulmonary artery is again uncommon, and associated cardiac malformations are usually tetralogy of fallot or septal defects rather than an aortic arch defect. Our case is a unique case in It’s associated congenital anomalies. He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch. The patient’s family has declined the surgical option, and he was managed conservatively and kept in close follow-up.

  16. 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.

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

    Science.gov (United States)

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

    2017-01-01

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

  18. Frequency and potential consequences of origin of the left vertebral artery (or the arteria thryoidea ima) directly from the aortic arch.

    Science.gov (United States)

    Zhang, Junlin; Guileyardo, Joseph M; Roberts, William C

    2016-10-01

    Described herein are findings in a 58-year-old man in whom necropsy disclosed origin of the left vertebral artery (or the arteria thryoidea ima) directly from the aortic arch. No functional consequences resulted. Study of previous publications disclosed the frequency of this anomaly in adults to be approximately 3.5%. Dissection has been reported to be more frequent in the left vertebral artery when it arises directly from the aorta than when it arises from the left subclavian artery.

  19. 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)

  20. Hybrid treatment of penetrating aortic ulcer

    International Nuclear Information System (INIS)

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

    2015-01-01

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

  1. 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 deformation during quiet standing and gait. Thirty subjects aged 20 to 32 years were included (15 with medial tibial stress syndrome and 15 controls). Navicular drop and medial longitudinal-arch deformation were measured during quiet standing with neutral and loaded foot using a ruler and digital photography....... Medial longitudinal-arch deformation was measured during walking gait using 3-dimensional gait analysis. Subjects with medial tibial stress syndrome demonstrated a significantly larger navicular drop (mean +/- 1 SD, 7.7 +/- 3.1 mm) and medial longitudinal-arch deformation (5.9 +/- 3.2 degrees) during...

  2. Fate of remnant sinuses of Valsalva in patients with bicuspid and trileaflet valves undergoing aortic valve, ascending aorta, and aortic arch replacement.

    Science.gov (United States)

    Milewski, Rita Karianna; Habertheuer, Andreas; Bavaria, Joseph E; Siki, Mary; Szeto, Wilson Y; Krause, Eric; Korutla, Varun; Desai, Nimesh D; Vallabhajosyula, Prashanth

    2017-08-01

    In patients presenting with aortic valvulopathy with concomitant ascending aortic aneurysm, surgical management of the sinus of Valsalva segment remains undefined, especially for moderately dilated aortic roots. In patients with this pathology undergoing aortic valve replacement with supracoronary ascending aorta replacement, we assessed the fate of the remnant preserved sinus of Valsalva segment stratified by aortic valve morphology and pathology. From 2002 to 2015, 428 patients underwent elective aortic valve replacement with supracoronary ascending aorta replacement. Patients were stratified on the basis of valvular morphology (bicuspid aortic valve [n = 254] and tricuspid aortic valve [n = 174]), valvular pathology (bicuspid aortic valve with aortic stenosis [n = 178], bicuspid aortic valve with aortic insufficiency [n = 76], tricuspid aortic valve with aortic stenosis [n = 61], tricuspid aortic valve with aortic insufficiency [n = 113]), and preoperative sinus of Valsalva dimensions (45 mm). Kaplan-Meier analysis revealed no significant difference in freedom from reoperation in tricuspid aortic valve versus bicuspid aortic valve (P = .576). Multivariable Cox regression model performed with sinus of Valsalva dimensions at baseline and follow-up as time-varying covariates did not adversely affect survival. A repeated-measure, mixed-effects model constructed to assess longitudinal sinus of Valsalva trends revealed that the retained sinus of Valsalva dimensions remain stable over long-term follow-up (discharge to ≥10 years), irrespective of valvular morphology/pathology (bicuspid aortic valve with aortic insufficiency, tricuspid aortic valve with aortic insufficiency, tricuspid aortic valve with aortic stenosis) and preoperative sinus of Valsalva groups (45 mm). In patients with nonaneurysmal sinuses of Valsalva undergoing aortic valve replacement with supracoronary ascending aorta replacement, the sinus segment can be preserved irrespective of

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

    Directory of Open Access Journals (Sweden)

    Sarah Morrissey, MD, MA, FRCSC

    2017-09-01

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

  4. 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. Copyright © 2014 Elsevier Inc. All rights reserved.

  5. Possible extracardiac predictors of aortic dissection in Marfan syndrome

    Science.gov (United States)

    2014-01-01

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

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

    Science.gov (United States)

    Cameron, Duke E; Vricella, Luca A

    2005-01-01

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

  7. Aortic valve sparing root surgery for Marfan syndrome.

    Science.gov (United States)

    Matalanis, George; Perera, Nisal K

    2017-11-01

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

  8. 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. Copyright © 2015 Elsevier Ltd. All rights reserved.

  9. Aortic Arch Calcification as a Predictor of Repeated Arteriovenous Fistula Failure within 1-Year in Hemodialysis Patients

    Directory of Open Access Journals (Sweden)

    Yit-Sheung Yap

    2017-01-01

    Full Text Available Objectives. The aim of the study was to identify the factors associated with repeated arteriovenous fistula (AVF failure within 1-year, especially the impact of aortic arch calcification (AAC on patency of AVF. Materials and Methods. We retrospectively assessed chest radiography in hemodialysis patients who had undergone initial AVF. The extent of AAC was categorized into four grades (0–3. The association between AAC grade, other clinical variables, and repeated failure of AVF was then analyzed by binary logistic regression analysis. Results. This study included 284 patients (158 males, mean age 61.7±13.1 years. Patients with higher AAC grade were older, had more frequently diabetes mellitus and cardiovascular disease, had lower diastolic blood pressure, and had higher corrected calcium and lower intact parathyroid hormone levels. In multivariate analysis, the presence of higher AAC grade (odds ratio (95% confidence interval: 2.98 (1.43–6.23; p=0.004, lower mean corrected calcium (p=0.017, and mean serum albumin level (p=0.008 were associated with repeated failure of AVF. Conclusions. The presence of higher AAC grade, lower mean corrected calcium and mean serum albumin level were independently associated with repeated AVF failure within 1 year in hemodialysis patients.

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

    DEFF Research Database (Denmark)

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

    2011-01-01

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

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

    OpenAIRE

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

    2015-01-01

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

  12. 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.

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

    Science.gov (United States)

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

    2014-01-01

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

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

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    Fernando Pivatto Júnior

    2015-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Chii-Shyan Lay

    2006-07-01

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

  16. Multiple Non-Syndromic Bilaterally Erupted Para Premolars in the Mandibular Arch: A Case Report

    Directory of Open Access Journals (Sweden)

    Amit Mehra

    2017-07-01

    Full Text Available Supernumerary teeth are the teeth present in addition to the normal set of teeth and are not uncommon in the general population. Presence of supernumerary teeth may affect the aesthetics of an individual and hence need appropriate management. It is however rare to find multiple supernumerary teeth which are not associated with any syndromes. Non-syndromic multiple supernumerary teeth have a predilection to occur in the mandibular premolar region. This paper reports an unusual case of an 18 year old boy with two completely erupted additional premolars on either side of the mandibular arch.

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

    OpenAIRE

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Hyung-Tae Sim

    2015-08-01

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

  19. Risk of 22q11.2 deletion in fetuses with right aortic arch and without intracardiac anomalies.

    Science.gov (United States)

    Perolo, A; De Robertis, V; Cataneo, I; Volpe, N; Campobasso, G; Frusca, T; Ghi, T; Prandstraller, D; Pilu, G; Volpe, P

    2016-08-01

    To assess the risk of 22q11.2 deletion in fetuses with a prenatal diagnosis of right aortic arch without intracardiac anomalies (RAA-no ICA). This was a retrospective study of all fetuses with RAA-no ICA diagnosed prenatally at three referral centers, between 2004 and 2014. A detailed sonographic examination was performed in each case, including visualization of the thymus and of the head and neck vessels to identify the presence of an aberrant left subclavian artery (ALSA). Karyotyping and fluorescence in situ hybridization analysis for diagnosis of 22q11.2 deletion were always offered either prenatally or postnatally. Clinical and echocardiographic examinations were performed in livebirths and a postmortem examination in cases of termination of pregnancy. During the study period, 85 fetuses were diagnosed prenatally with RAA-no ICA. Genetic or clinical data were not available for three cases and these were excluded from analysis. 22q11.2 deletion was found in 7/82 cases (8.5% (95% CI, 3.8-17.3%)). The thymus was small or non-visualized in all seven cases and additional abnormal sonographic findings were present in four. 22q11.2 deletion is present in a clinically significant proportion of fetuses with a prenatal diagnosis of RAA-no ICA. In such cases, a detailed sonographic examination, with assessment of the thymus in particular, may be useful to further define the level of risk for 22q11.2 deletion. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

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

    Science.gov (United States)

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

    2015-10-15

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

  1. 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

  2. Brief review on systematic hypothermia for the protection of central nervous system during aortic arch surgery: a double-sword tool?

    Directory of Open Access Journals (Sweden)

    Parissis Haralabos

    2011-11-01

    Full Text Available Abstract Antegrade selective cerebral perfusion in conjunction with hypothermia attenuate postoperative neurological injury, which in turn still remains the main cause of mortality and morbidity following aortic arch surgery. Hypothermic circulatory arrest however could be a useful tool during arch surgery, surgery for chronic thromboembolic disease, air on the arterial line during CPB, during cavotomy for extraction of renal cell carcinoma with level IV extension, or when dealing with difficult trauma to the SVC or IVC. Cerebral protective effects with hypothermic procedures including inhibition of neuron excitation, and discharge of excitable amino acids, and thereby, prevention of an increase in intercellular calcium ions, hyperoxidation of lipids in cell membranes, and free radical production. The authors are briefly discussing the fundamental principles of using hypothermia as an adjunct tool of the cardiothoracic surgeon's practice. The relationship between temperature, flow, metabolic requirements and adverse effects is addressed.

  3. 5B.05: MARFAN SYNDROME: ASSESSMENT OF AORTIC DISSECTION RISK BY ANALYSIS OF AORTIC VISCOELASTIC PROPERTIES.

    Science.gov (United States)

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

    2015-06-01

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

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

    Science.gov (United States)

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

    2011-11-01

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

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

    DEFF Research Database (Denmark)

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

    2018-01-01

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

  6. 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....

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

    NARCIS (Netherlands)

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

    2015-01-01

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

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

    NARCIS (Netherlands)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Sirisha Nandipati

    2013-12-01

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

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

    Science.gov (United States)

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

    2017-07-01

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

  11. Two-stage hybrid treatment strategy for an adult patient with aortic arch coarctation, poststenotic aneurysm, and hypoplastic left subclavian artery: A case report.

    Science.gov (United States)

    Pu, Xiao-Bo; Chen, Shi-Jian; Chen, Mao; Feng, Yuan

    2017-12-01

    Coarctation of aorta in adulthood is usually complicated by other cardiovascular anomalies, posing great technical challenge for intervention. Here, we report an extremely rare case of aortic arch coarctation combined with a poststenotic biloculated calcified aneurysm and hypoplastic left subclavian artery. First, an extra-anatomic bypass was established, along with narrowing of aorta just proximal and distal to the aneurysm. While the bypass graft significantly relieved trans-coarctation gradient, the latter procedure decreased intra-aneurysm pressure and created landing zones for aneurysm occlusion. Six months later, 2 muscular ventricular septal defect occluders were deployed at the proximal and distal orifice of the aneurysm. Follow-up computed tomography angiography confirmed the absence of contrast leakage into aneurysm. A 2-stage hybrid approach described here appears to be feasible, safe, and associated with favorable clinical outcomes in the treatment of complicated aortic coarctation and poststenotic aneurysm.

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

    Science.gov (United States)

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

    2009-10-01

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

  13. Pathogenetic Basis of Aortopathy and Aortic Valve Disease

    Science.gov (United States)

    2018-02-19

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

  14. 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

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

    Science.gov (United States)

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

    2018-01-29

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

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

    NARCIS (Netherlands)

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

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

  17. Platypnea-Orthodeoxia Syndrome after Transcatheter Aortic Valve Implantation

    Directory of Open Access Journals (Sweden)

    Andrew K. Roy

    2016-01-01

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

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

    Science.gov (United States)

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

    2007-12-01

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

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

    Science.gov (United States)

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

    2017-11-07

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

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

    Directory of Open Access Journals (Sweden)

    Ling Lee

    Full Text Available Marfan syndrome (MFS is an autosomal-dominant disorder of connective tissue caused by mutations in the fibrillin-1 (FBN1 gene. Mortality is often due to aortic dissection and rupture. We investigated the structural and functional properties of the heart and aorta in a [Fbn1C1039G/+] MFS mouse using high-resolution ultrasound (echo and optical coherence tomography (OCT. Echo was performed on 6- and 12-month old wild type (WT and MFS mice (n = 8. In vivo pulse wave velocity (PWV, aortic root diameter, ejection fraction, stroke volume, left ventricular (LV wall thickness, LV mass and mitral valve early and atrial velocities (E/A ratio were measured by high resolution echocardiography. OCT was performed on 12-month old WT and MFS fixed mouse hearts to measure ventricular volume and mass. The PWV was significantly increased in 6-mo MFS vs. WT (366.6 ± 19.9 vs. 205.2 ± 18.1 cm/s; p = 0.003 and 12-mo MFS vs. WT (459.5 ± 42.3 vs. 205.3 ± 30.3 cm/s; p< 0.0001. PWV increased with age in MFS mice only. We also found a significantly enlarged aortic root and decreased E/A ratio in MFS mice compared with WT for both age groups. The [Fbn1C1039G/+] mouse model of MFS replicates many of the anomalies of Marfan patients including significant aortic dilation, central aortic stiffness, LV systolic and diastolic dysfunction. This is the first demonstration of the direct measurement in vivo of pulse wave velocity non-invasively in the aortic arch of MFS mice, a robust measure of aortic stiffness and a critical clinical parameter for the assessment of pathology in the Marfan syndrome.

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

    OpenAIRE

    Phowthongkum, Prasit

    2010-01-01

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

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

    Science.gov (United States)

    Phowthongkum, Prasit

    2010-01-01

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

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

    Science.gov (United States)

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

    2015-03-01

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

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

    Science.gov (United States)

    Nawata, Kan; Morota, T

    2009-10-01

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

  5. Gothic aortic arch and cardiac mechanics in young patients after arterial switch operation for d-transposition of the great arteries.

    Science.gov (United States)

    Di Salvo, Giovanni; Bulbul, Ziad; Pergola, Valeria; Issa, Ziad; Siblini, Ghassan; Muhanna, Nisreen; Galzerano, Domenico; Fadel, Bahaa; Al Joufan, Mansour; Al Fayyadh, Majid; Al Halees, Zohair

    2017-08-15

    In patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associated with abnormal aortic bio-elastic properties. GA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1- the occurrence of GA in a large sample of patients after ASO; 2- the association between GA and aortic bio-elastic properties; and 3- the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE). We studied one hundred and five asymptomatic patients, who have undergone first stage ASO for d-TGA, with normal left ventricular ejection fraction (LVEF ≥53%). Forty-six (44%) patients showed a GA (mean age 11.5±7.2years, 26 males) while fifty-nine (56%) patients (mean age 9.6±6.7years, 37 males) did not present GA. The two groups were comparable for age, sex, BSA, and office blood pressure values. In group GA aortic root was significantly dilated (27.4±7.5mm vs. 21.2±6.9mm, p<0.0001), aortic stiffness index (Group GA=1.8±1.2 vs. 1.4±0.7, p=0.025) was significantly increased, left atrial volume was larger (p=0.0145), global longitudinal strain (Group GA=-18.4±2.5% vs. -20.1±3.3%, p=0.012) and basal LV longitudinal strains (Group GA=-16.9±4.8% vs. -20.4±7.0%, p=0.013) were significantly reduced. After ASO the presence of a GA is associated with a significantly dilated aortic root, stiffer aortic wall, larger left atrial volume, and worse LV longitudinal systolic deformations, well known predictors of cardiovascular morbidity and mortality. Copyright © 2017 Elsevier B.V. All rights reserved.

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

    NARCIS (Netherlands)

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

    2016-01-01

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

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

    NARCIS (Netherlands)

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

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Iuliano Marialetizia

    2007-12-01

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

  9. Prenatal Diagnosis of Fetal Interrupted Aortic Arch Type A by Two-Dimensional Echocardiography and Four-Dimensional Echocardiography with B-Flow Imaging and Spatiotemporal Image Correlation.

    Science.gov (United States)

    Zhang, Dongyu; Zhang, Ying; Ren, Weidong; Sun, Feifei; Guo, Yajun; Sun, Wei; Wang, Yu; Huang, Liping; Cai, Ailu

    2016-01-01

    Fetal interrupted aortic arch (IAA) is a rare cardiac anomaly and its prenatal diagnosis is challenging. The purpose of our report is to evaluate the use of two-dimensional echocardiography (2DE) and 4D echocardiography with B-flow imaging and spatiotemporal image correlation (4D BF-STIC) in detecting IAA type A (IAA-A). Twenty-three cases of confirmed IAA-A identified by fetal echocardiography were involved in the study. The fetal echocardiography image data were reviewed to analyze the ratio of right ventricle to left ventricle (RV/LV) diameter, the ratio of main pulmonary artery to ascending aorta (MPA/AAO) diameter, and the correlation of RV/LV diameter ratio and size of ventricular septal defect (VSD). 4D BF-STIC was performed in 21 fetuses using the sagittal view (4D BF-STIC-sagittal) and the four-chamber view (4D BF-STIC-4CV) as initial planes of view. An additional 183 normal fetuses were also included in our study. RV/LV and MPA/AAO ratios were calculated and compared with that of IAA-A fetuses. Fetal 2DE, 4D BF-STIC-sagittal, and 4D BF-STIC-4CV were used to visualize the aortic arch and its associated neck vessels. Six subgroups were evaluated according to gestational age. Fetal 2DE, 4D BF-STIC-sagittal, and 4D BF-STIC-4CV made the correct prenatal diagnosis of IAA-A in 19/23 (82.6%), 14/21 (66.7%), and 19/21 (90.5%) of patients, respectively. A significantly enlarged MPA combined with symmetric ventricles was found in the IAA-A fetuses, while the size of the VSD was negatively correlated with RV/LV ratio. 4D BF-STIC-sagittal and 4D BF-STIC-4CV were better than traditional 2D ultrasound in detecting the aortic arch and neck vessels between 17 and 28 gestational weeks and 29 to 40 gestational weeks in normal fetuses. It is demonstrated that IAA-A could be diagnosed by traditional fetal echocardiography, while 4D technique could better display the anatomic structure and the spatial relationships of the great arteries. Use of volume reconstruction may

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

    NARCIS (Netherlands)

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

    2012-01-01

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

  11. 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.

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

    International Nuclear Information System (INIS)

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

    2014-01-01

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

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

    Science.gov (United States)

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

    2017-11-01

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

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

    Science.gov (United States)

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

    2017-04-01

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

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

    Science.gov (United States)

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

    2014-10-01

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

  16. Arterial access through the right subclavian artery in surgery of the aortic arch improves neurologic outcome and mid-term quality of life.

    Science.gov (United States)

    Immer, Franz F; Moser, Barbara; Krähenbühl, Eva S; Englberger, Lars; Stalder, Mario; Eckstein, Friedrich S; Carrel, Thierry

    2008-05-01

    We have shown that selective antegrade cerebral perfusion improves mid-term quality of life in patients undergoing surgical repair for acute type A aortic dissection and aortic aneurysms. The aim of the study was to assess the impact of continuous cerebral perfusion through the right subclavian artery on immediate outcome and quality of life. Perioperative data of 567 consecutive patients who underwent surgery of the aortic arch using deep hypothermic circulatory arrest have been analyzed. Patients were divided into three groups, according to the management of cerebral protection. Three hundred eighty-seven patients (68.3%) had deep hypothermic circulatory arrest with pharmacologic protection with pentothal only, 91 (16.0%) had selective antegrade cerebral perfusion and pentothal, and 89 (15.7%) had continuous cerebral perfusion through the right subclavian artery and pentothal. All in-hospital data were assessed, and quality of life was analyzed prospectively 2.4 +/- 1.2 years after surgery with the Short Form-36 Health Survey Questionnaire. Major perioperative cerebrovascular injuries were observed in 1.1% of the patients with continuous cerebral perfusion through the right subclavian artery, compared with 9.8% with selective antegrade cerebral perfusion (p life after an arrest time between 30 and 50 minutes with continuous cerebral perfusion through the right subclavian artery was significantly better than selective antegrade cerebral perfusion (90.2 +/- 12.1 versus 74.4 +/- 40.7; p = 0.015). Continuous cerebral perfusion through the right subclavian artery improves considerably perioperative brain protection during deep hypothermic circulatory arrest. Irreversible perioperative neurologic complications can be significantly reduced and duration of deep hypothermic circulatory arrest can be extended up to 50 minutes without impairment in quality of life.

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

    Science.gov (United States)

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

    2008-06-01

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

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

    Science.gov (United States)

    2013-01-01

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

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

    Science.gov (United States)

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

    2011-08-01

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

  20. Disturbed morphogenesis of cardiac outflow tract and increased rate of aortic arch anomalies in the offspring of diabetic rats.

    Science.gov (United States)

    Molin, Daniël G M; Roest, Pauline A M; Nordstrand, Hanna; Wisse, Lambertus J; Poelmann, Robert E; Eriksson, Ulf J; Gittenberger-De Groot, Adriana C

    2004-12-01

    Maternal diabetes (MD) is a risk factor for offspring to develop cardiovascular anomalies; this is of growing clinical concern since the number of women in childbearing age with compromised glucose homeostasis is increasing. Hyperglycemia abrogates cardiovascular development in vitro; however, a link to cardiovascular defects in diabetic offspring remains to be investigated. We have studied cardiovascular development in offspring of MD rats by examining serial histological sections of GD 12.0-18.0 offspring. Development of pharyngeal arch artery malformations was analyzed and related to intracardiac anomalies. Pharyngeal arch artery and intracardiac defects were present in 27 of 37 MD GD 13.0-18.0 offspring. Early sixth arch arteries showed abrogated arteriogenesis, whereas fourth arch artery defects developed as a result of abnormal remodeling. Morphometrical analysis showed increased apoptosis in regressing artery segments and reduced apoptosis in persisting artery segments. Double outlet right ventricle with infundibular stenosis (tetralogy of Fallot) was predominantly found in combination with sixth artery defects and pulmonary atresia. As confirmed by morphometric analysis and three-dimensional (3D)-reconstructions, outflow tract defects coincided with endocardial cushion hypoplasia. Cases with teratology of Fallot additionally showed a shorter outflow tract. No relation with apoptosis or disturbed neural crest cell migration was found. Our data uniquely demonstrate mechanistic differences involved in the development of sixth and fourth artery anomalies. Whereas increased apoptosis induces fourth artery anomalies, pulmonary outflow obstruction abrogates sixth artery differentiation independent of apoptosis. The model presented allows analysis of diabetic conditions on cardiovascular development in vivo, essential for elucidating this teratology.

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

    NARCIS (Netherlands)

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

    2005-01-01

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

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

    NARCIS (Netherlands)

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

    2005-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1995-06-01

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

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

    Science.gov (United States)

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

    2018-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Mandana Master

    2012-01-01

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

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

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    Rayan Jo Rachwan

    2017-09-01

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

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

    Science.gov (United States)

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

    2005-08-24

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

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

    Science.gov (United States)

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

    2015-04-07

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

  9. Impact of methodology and the use of allometric scaling on the echocardiographic assessment of the aortic root and arch: a study by the Research and Audit Sub-Committee of the British Society of Echocardiography.

    Science.gov (United States)

    Oxborough, David; Ghani, Saqib; Harkness, Allan; Lloyd, Guy; Moody, William; Ring, Liam; Sandoval, Julie; Senior, Roxy; Sheikh, Nabeel; Stout, Martin; Utomi, Victor; Willis, James; Zaidi, Abbas; Steeds, Richard

    2014-09-01

    The aim of the study is to establish the impact of 2D echocardiographic methods on absolute values for aortic root dimensions and to describe any allometric relationship to body size. We adopted a nationwide cross-sectional prospective multicentre design using images obtained from studies utilising control groups or where specific normality was being assessed. A total of 248 participants were enrolled with no history of cardiovascular disease, diabetes, hypertension or abnormal findings on echocardiography. Aortic root dimensions were measured at the annulus, the sinus of Valsalva, the sinotubular junction, the proximal ascending aorta and the aortic arch using the inner edge and leading edge methods in both diastole and systole by 2D echocardiography. All dimensions were scaled allometrically to body surface area (BSA), height and pulmonary artery diameter. For all parameters with the exception of the aortic annulus, dimensions were significantly larger in systole (P<0.05). All aortic root and arch measurements were significantly larger when measured using the leading edge method compared with the inner edge method (P<0.05). Allometric scaling provided a b exponent of BSA(0.6) in order to achieve size independence. Similarly, ratio scaling to height in subjects under the age of 40 years also produced size independence. In conclusion, the largest aortic dimensions occur in systole while using the leading edge method. Reproducibility of measurement, however, is better when assessing aortic dimensions in diastole. There is an allometric relationship to BSA and, therefore, allometric scaling in the order of BSA(0.6) provides a size-independent index that is not influenced by the age or gender.

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

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    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.

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

    Directory of Open Access Journals (Sweden)

    Christopher Yu

    2018-03-01

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

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

    Science.gov (United States)

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

    2018-03-01

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

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

    Science.gov (United States)

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

    2016-02-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2017-04-15

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

  15. 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.

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

    Directory of Open Access Journals (Sweden)

    Hai-Ou Li

    2015-01-01

    Full Text Available 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 of DSCT 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, 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.

  17. Persistent right aortic arch with an aberrant left subclavian artery, Kommerell’s diverticulum and bicarotid trunk in a 3-year-old cat

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    Dylan Shannon

    2015-10-01

    Full Text Available Case summary A 3-year-old male, neutered, domestic shorthair cat with a history of chronic regurgitation since being obtained as a kitten was presented for weight loss and regurgitation of all ingested food. The cat was in poor body condition and had a firm swelling in the ventral neck at the time of presentation. Thoracic radiographs showed severe dilation of the entire cervical and cranial intrathoracic esophagus to the level of the heart base. Computed tomographic angiography (CTA showed a persistent right aortic arch with an aberrant left subclavian artery and severe dilation of the cervical and intrathoracic esophagus cranial to the heart base. CTA also showed a bicarotid trunk and Kommerell’s diverticulum to be present, which are rare vascular structures in the cat. Esophagoscopy showed esophageal dilation and multiple compact trichobezoars obstructing the esophagus. Removal of the obstructing trichobezoars resulted in resolution of clinical signs, and the cat was able to drink water and eat a canned food slurry without regurgitation. Surgical correction was not pursued. Relevance and novel information Vascular ring anomaly (VRA should be considered in all cats with a history of regurgitation, regardless of their age at the time of presentation. CTA is a valuable diagnostic imaging procedure that allows differentiation of a VRA from other causes of esophageal obstruction and provides information about the VRA that can be used to determine amenability to surgical correction.

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

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    Savina Mannarino

    2015-11-01

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

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

    Science.gov (United States)

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

    2014-02-01

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

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

    Science.gov (United States)

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

    2015-07-01

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

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

    Science.gov (United States)

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

    2017-09-01

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

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

    Science.gov (United States)

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

    2015-12-01

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

  3. 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

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

    Science.gov (United States)

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

    2018-03-22

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

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

    NARCIS (Netherlands)

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

    1998-01-01

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

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

    NARCIS (Netherlands)

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

    2012-01-01

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

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

    NARCIS (Netherlands)

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

    2015-01-01

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

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

    OpenAIRE

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

    1981-01-01

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

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

    Science.gov (United States)

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

    2017-06-01

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

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

    Science.gov (United States)

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

    2014-05-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2011-09-01

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

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

    Science.gov (United States)

    Smith, Katherine; Gros, Bernard

    2017-05-01

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

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

    Science.gov (United States)

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

    2018-02-01

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

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

    Science.gov (United States)

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

    2013-09-10

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

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

    OpenAIRE

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

    1985-01-01

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

  17. 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.

  18. 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

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

    Science.gov (United States)

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

    2017-12-15

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

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

    Science.gov (United States)

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

    2010-01-01

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

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

    Science.gov (United States)

    2014-01-01

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

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

    International Nuclear Information System (INIS)

    Zhi Aihua; Dai Ruping; Jiang Shiliang

    2007-01-01

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

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

    DEFF Research Database (Denmark)

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

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

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

    Science.gov (United States)

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

    2016-02-01

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

  5. Management of neonates with right-sided aortic arch and esophageal atresia: International survey on IPEG AND ESPES members´ experience.

    Science.gov (United States)

    Aguilera-Pujabet, Monserrat; Gahete, Jose Andres Molino; Guillén, Gabriela; López-Fernández, Sergio; Martin-Giménez, Marta Patricia; Lloret, Josep; López, Manuel

    2017-11-23

    The optimum surgical approach of neonates with right-sided aortic arch (RAA) and esophageal atresia (EA)/tracheoesophageal fistula (TEF) is still an unsolved question. In order to propose an operative algorithm in the era of endoscopic surgery, we performed an international survey to know the current practice between pediatric endoscopic surgeons. Two of the most important societies in endoscopic pediatric surgery were queried: the International Pediatric Endosurgery Group (IPEG) and the European Society of Paediatric Endoscopic Surgeons (ESPES). During December 2016, an anonymous online-based survey was sent to all IPEG and ESPES members, collecting data regarding perioperative management and surgical repair of EA/TEF with RAA. 144 surgeons from 23 countries completed the questionnaire. 69.2% of respondents were IPEG members, 30.8% were ESPES members. 71.5% of members who answered the survey had more than 10years of surgical experience. A preoperative echocardiography was almost uniformly performed (93.1%). 31.9% of the surveyed surgeons had never treated an EA/TEF with RAA. The remaining 98 surveyed surgeons had managed 279 cases of EA/TEF with RAA. Thoracotomy was considered the preferred approach for 54.2% of the surgeons, and 51.9% chose a right-sided approach. When RAA was an intraoperative finding, 76% would perform a contralateral thoracotomy if difficulties arose. Thoracoscopy was preferred by 45.8% of surgeons. If RAA was suspected preoperatively, 63.1% preferred to attempt a left-sided thoracoscopy and only 24.2% would change their approach to a thoracotomy. If RAA was an intraoperative finding and a safe surgical repair could not be achieved through right-sided thoracoscopy, 51.5% of them chose to perform a left sided thoracoscopy, rather than convert to thoracotomy. Preoperative echocardiography performed by experienced examiners helps in surgical planning. Preoperative diagnosis of RAA should not discourage thoracoscopic repair, which is increasingly

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

    Science.gov (United States)

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

    2008-06-01

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

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

    International Nuclear Information System (INIS)

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

    1987-01-01

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

  8. Long-term outcomes of frozen elephant trunk for type A aortic dissection in patients with Marfan syndrome.

    Science.gov (United States)

    Ma, Wei-Guo; Zhang, Wei; Zhu, Jun-Ming; Ziganshin, Bulat A; Zhi, Ai-Hua; Zheng, Jun; Liu, Yong-Min; Elefteriades, John A; Sun, Li-Zhong

    2017-10-01

    The use of the frozen elephant trunk (FET) technique for repair of type A aortic dissection (TAAD) in Marfan syndrome (MFS) is controversial. We seek to evaluate the efficacy of FET and total arch replacement (TAR) for TAAD in patients with MFS. The early and long-term outcomes were analyzed for 106 patients with MFS (mean age, 34.5 ± 9.7 years) undergoing FET + TAR for TAAD. Operative mortality was 6.6% (7 of 106). Spinal cord injury and stroke occurred in 1 patient each (0.9%), and reexploration for bleeding occurred in 6 patients (5.7%). Extra-anatomic bypass was the sole risk factor for operative mortality and morbidity (odds ratio [OR], 7.120; 95% confidence interval [CI], 1.018-49.790; P = .048). Follow-up was complete in 97.0% (96 of 99), averaging 6.3 ± 2.8 years. Late death occurred in 17 patients. Patients with acute TAAD were less prone to late death than those with chronic TAAD (OR, 0.112; 95% CI, 0.021-0.587; P = .048). Twelve patients required late reoperation, including thoracoabdominal aortic repair in 8, thoracic endovascular aortic repair for distal new entry in 3, and coronary anastomotic repair in 1. At 5 years, survival was 86.6% (95% CI, 77.9%-92.0%) and freedom from reoperation was 88.8% (95% CI, 80.1%-93.4%), and at 8 years, survival was 74.1% (95% CI, 61.9%-83.0%) and freedom from reoperation was 84.2% (95% CI, 72.4%-91.2%). In competing risks analysis, mortality was 4% at 5 years, 18% at 8 years, and 25% at 10 years; the respective rates of reoperation were 10%, 15%, and 15%; and the respective rates of survival without reoperation were 86%, 67%, and 60%. Survival was significantly higher in patients who underwent root procedures during FET + TAR (P = .047). Risk factors for reoperation were days from diagnosis to surgery (OR, 1.160; 95% CI, 1.043-1.289; P = .006) and Bentall procedure (OR, 12.012; 95% CI, 1.041-138.606; P = .046). The frozen elephant trunk and total arch replacement procedure can be safely performed

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

    International Nuclear Information System (INIS)

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

    2013-01-01

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

  10. 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.

  11. High arch

    Science.gov (United States)

    ... pes cavus. High arch is the opposite of flat feet . ... High foot arches are much less common than flat feet. They are more likely to be caused by a bone (orthopedic) or nerve (neurological) condition. Unlike ...

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

    Science.gov (United States)

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

    2010-09-01

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

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

    Science.gov (United States)

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

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Lu-Hang Liu

    2018-04-01

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

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

    Science.gov (United States)

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

    2011-01-01

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

  16. 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.

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

    Directory of Open Access Journals (Sweden)

    Rosina De Cario

    2014-01-01

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

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

    Science.gov (United States)

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

    2013-05-01

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

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

    Science.gov (United States)

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

    2014-09-01

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

  20. 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...

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

    Science.gov (United States)

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

    2011-01-01

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

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

    Science.gov (United States)

    Yakut, Kahraman; Erdoğan, İlkay

    2017-03-01

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

  3. Re-interventions on the thoracic and thoracoabdominal aorta in patients with Marfan syndrome.

    Science.gov (United States)

    Schoenhoff, Florian S; Carrel, Thierry P

    2017-11-01

    The advent of multi-gene panel genetic testing and the discovery of new syndromic and non-syndromic forms of connective tissue disorders have established thoracic aortic aneurysms as a genetically mediated disease. Surgical results in patients with Marfan syndrome (MFS) provide an important benchmark for this patient population. Prophylactic aortic root surgery prevents acute dissection and has contributed to the improved survival of MFS patients. In the majority of patients, re-interventions are driven by a history of dissection. Patients undergoing elective root repair have a low risk for re-interventions on the root itself. Experienced centers have results after valve-sparing procedures at 10 years comparable with those seen after a modified Bentall procedure. In patients where only the ascending aorta was replaced during the initial surgery, re-intervention rates are high as the root continues to dilate. The fate of the aortic arch in MFS patients presenting with dissection is strongly correlated with the extent of the initial surgery. Not replacing the entire ascending aorta and proximal aortic arch results in a high rate of re-interventions. Nevertheless, the additional burden of replacing the entire aortic arch during emergent proximal repair is not very well defined and makes comparisons with patients undergoing elective arch replacement difficult. Interestingly, replacing the entire aortic arch during initial surgery for acute dissection does not protect from re-interventions on downstream aortic segments. MFS patients suffering from type B dissection have a high risk for re-interventions ultimately leading up to replacement of the entire thoracoabdominal aorta even if the dissection was deemed uncomplicated by conventional criteria. While current guidelines do not recommend the implantation of stent grafts in MFS patients, implantation of a frozen-elephant-trunk to create a stable proximal landing zone for future endovascular or open procedures has

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

    Science.gov (United States)

    Zhuraev, R K; Zerbino, D D

    2014-01-01

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

  5. Aortic Aneurysm Statistics

    Science.gov (United States)

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

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

    Science.gov (United States)

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

    2015-08-01

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

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

    Science.gov (United States)

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

    2012-03-01

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

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

    Science.gov (United States)

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

    2015-01-01

    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

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

    Science.gov (United States)

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

    2018-04-01

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

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

    Directory of Open Access Journals (Sweden)

    Jong Uk Lee

    2016-04-01

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

  11. 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.

  12. PHACE(S syndrome: Report of a case with new ocular and systemic manifestations

    Directory of Open Access Journals (Sweden)

    Raheleh Assari

    2017-06-01

    Conclusions: To our knowledge, this report was the first report of a pattern like Brown’s syndrome (may be called apparent Brown’s syndrome and the second report of the congenital glaucoma in a case of PHACE(S syndrome. In addition, the anomalous origin of the vertebral artery from the aortic arch has not been reported in the PHACE(S syndrome. Thus, the clinicians should perform the glaucoma work-up for each patient with this syndrome.

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

    Science.gov (United States)

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

    2014-05-01

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

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

    Science.gov (United States)

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

    2017-04-19

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

  15. Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

    Science.gov (United States)

    Qureshi, Muhammad Yasir; Burkhart, Harold M; Julsrud, Paul; Cetta, Frank

    2014-12-01

    Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

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

    Science.gov (United States)

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

    2015-05-01

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

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

    Science.gov (United States)

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

    2017-03-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2011-06-01

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

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

    Science.gov (United States)

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

    2018-01-12

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

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

    Science.gov (United States)

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

    2017-08-08

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

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

    Directory of Open Access Journals (Sweden)

    María E. Soto

    2018-02-01

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

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

    Science.gov (United States)

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

    2007-02-01

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

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

    Science.gov (United States)

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

    2014-07-01

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

  5. Consecutive operative procedures in patients with Marfan syndrome up to 28 years after initial aortic root surgery.

    Science.gov (United States)

    Puluca, Nazan; Burri, Melchior; Cleuziou, Julie; Krane, Markus; Lange, Rüdiger

    2018-02-28

    Most patients (75%) with Marfan syndrome present with aortic root dilatation that may require surgical intervention. However, associated cardiovascular disorders are not limited to the aortic root. These patients frequently require consecutive operations on the remaining thoracic aorta or the heart valves. Our intent was to characterize the spectrum of such procedures. Data from all patients with Marfan syndrome undergoing aortic root surgery at our centre between 1988 and 2016 were analysed retrospectively. Overall, 73 patients (26 women) were selected for the study. The median age at 1st operation was 30 years (3-68 years). Indications for aortic root surgery were aneurysm (78%) and dissection (22%). Initially, 33 Bentall procedures and 40 valve-sparing root replacement procedures were performed, with a 97% rate of follow-up completion. The median follow up was 8 years (0-28 years). Survival at 1, 10 and 15 years was 100%, 85% and 82%, respectively. During follow-up monitoring, 48 subsequent procedures were performed in 33 patients (aorta, 23; aortic valve, 11; mitral valve, 7 and combined procedures, 7). The 30-day mortality rate after subsequent procedures was 4.2%. Freedom from subsequent operation and death (combined end-point) after 5, 10 and 15 years was 70%, 53% and 34%, respectively. After the initial surgery, subsequent procedures required in the setting of Marfan syndrome most often involve the remaining native aorta, followed by the aortic and mitral valves. The continued need for additional operative procedures remains high, even decades after the initial surgeries are undertaken. Therefore, long-term patient monitoring at specialized centres is imperative.

  6. Cardiovascular Malformations in CHARGE Syndrome with DiGeorge Phenotype: Two Case Reports

    OpenAIRE

    Yasuda, Kazushi; Morihana, Eiji; Fusazaki, Naoki; Ishikawa, Shiro

    2016-01-01

    Both CHARGE syndrome and DiGeorge anomaly are frequently accompanied by cardiovascular malformations. Some specific cardiovascular malformations such as interrupted aortic arch type B and truncus arteriosus are frequently associated with 22q11.2 deletion syndrome, while conotruncal defects and atrioventricular septal defects are overrepresented in patients with CHARGE syndrome. CHD7 gene mutation is identified in approximately two-thirds of patients with CHARGE syndrome, and chromosomal micro...

  7. Abdominal compartment syndrome caused by ruptured abdominal aortic aneurysm in vena cava

    Directory of Open Access Journals (Sweden)

    Filipović Aleksandar

    2006-01-01

    Full Text Available Background. Abdominal compartment syndrome (ACS is a rapid increase in intra-abdominal pressure associated with multi-organs dysfunction. It is caused mostly by abdominal bleeding und massive volume compensation. Case report. We reported a 76-year-old patient admitted to the hospital with aortic abdominal aneurysm, 13.7 cm in diameter, ruptured in vena cava, which caused intraabdominal hypertension, the liver and kidney dysfunction, as well as circulation, respiration and metabolic disorders. Intraabdominal pressure was measured by bladder manometry. Central venous pressure and systemic arterial pressure were monitored continuously. Clinical signs were thrill and typical abdominal bruit. Aorto-caval fistula was diagnosed by the use of contrast computerized tomography. Caval endoaneurysmatic suture and aortobiiliac bypass with 18 × 9 mm Dacron prothesis were performed. Haemodynamic changes were mostly corrected during the surgery. The complete correction of haemodynamics, liver, kidney, respiration and metabolic changes was established in the next few weeks. Conclusion. The ACS was caused by rupture of abdominal aortic aneurysm in vena cava followed by edema of the abdominal organs, retroperitoneum, abdominal wall and ascites. Caval endoaneurysmatic suture and aortobiiliac bypass with 18 × 9 mm Dacron prothesis solved aortocaval fistula as well as all the organs and metabolic dysfunctions caused by ACS.

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

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    Hung Yi Chen

    2015-06-01

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

  9. 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.

  10. Distant downstream steady-state flow studies of a mechanical heart valve: PIV study of secondary flow in a model aortic arch

    Science.gov (United States)

    Fix, Brandon R.; Popma, Christopher J.; Bulusu, Kartik V.; Plesniak, Michael W.

    2013-11-01

    Each year, hundreds of thousands of aortic and mitral heart valves are replaced with prosthetic valves. In efforts to develop a valve that does not require lifelong anticoagulation therapy, previous experimental research has been devoted to analyzing the hemodynamics of various heart valve designs, limited to the flow up to only 2 diameters downstream of the valve. Two-component, two-dimensional (2C-2D) particle image velocimetry (PIV) was used in this study to examine secondary flow velocity fields in a curved tube modeling an aorta at five locations (0-, 45-, 90-, 135-, 180-degrees). A bileaflet valve, opened to 30-, 45-, and 59-degrees, and one (no-valve) baseline condition were examined under three steady flow inflows (Re = 218, 429, 634). In particular, variations in the two-dimensional turbulent shear stresses at each cross sectional plane were analyzed. The results suggest that bileaflet valves in the aortic model produce significant turbulence and vorticity up to 5.5 downstream diameters, i.e. up to the 90-degrees location. Expanding this research towards aortic heart valve hemodynamics highlights a need for additional studies extending beyond the typical few diameters downstream to fully characterize valvular function. Supported by the NSF Grant No. CBET- 0828903 and GW Center for Biomimetics and Bioinspired Engineering.

  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. Copyright © 2013 Wiley Periodicals, Inc.

  12. Pseudoaneurisma de aorta como causa de síndrome da veia cava superior: relato de caso Aortic pseudoaneurysm as cause of superior vena cava syndrome: a case report

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    Katsuro Harada Júnior

    2012-09-01

    Full Text Available A síndrome da veia cava superior representa o conjunto de sinais e sintomas decorrentes da sua obstrução. A síndrome tem como principais etiologias tumores malignos, como o carcinoma broncogênico, o linfoma e a metástase mediastinal. O câncer de pulmão é responsável por 80% dos casos, os linfomas mediastinais por 15%, e 5% correspondem às demais causas. Este relato de caso objetiva apresentar um caso incomum dessa síndrome, ocorrido em um paciente do sexo masculino após ferimento penetrante no tórax, resultando em pseudoaneurisma de arco aórtico e a síndrome da veia cava superior.The superior vena cava syndrome represents the set of signs and symptoms resulting from obstruction of superior vena cava. The syndrome has as main causes malignant tumors such as bronchogenic carcinoma, lymphoma and mediastinal metastases. Lung cancer accounts for 80% of cases, mediastinal lymphomas by 15% and 5% correspond to other causes. This case report aims to present an unusual case of this syndrome, which occurred in a male patient after penetrating wound in the chest, which developed a pseudoaneurysm of the aortic arch and superior vena cava syndrome.

  13. [Anesthetic Management of a Patient Complicated with Marfan Syndrome and Suffering from Stanford Type A Aortic Dissection during Pregnancy].

    Science.gov (United States)

    Uozaki, Nako; Mizuno, Kaori; Shiraishi, Yoshito; Doi, Matsuyuki; Sato, Shigehito

    2015-04-01

    We report a case of a 36-year-old woman at 34 weeks of gestation complicated with Marfan syndrome who underwent Bentall type aortic replacement surgery due to Stanford type A aortic dissection after undergoing caesarean section. Since this patient exhibited severe hypotension before coming to the operating room, it was very difficult to determine whether the cardiac surgery or caesarean section should be performed first. In this case, the caesarean section was performed first, followed by Bentall's surgery. Although intra-aortic balloon pumping and percutaneous cardiopulmonary support were required after weaning from the cardiopulmonary bypass, she was discharged on post-operative day (POD) 40 and the baby was discharged on POD 60, without signs of cerebral palsy. Unfortunately, this patient died on POD 57, due to heart failure. We discuss how to determine the priority of surgeries for patients who require emergency surgery for cardiovascular disease during pregnancy.

  14. Computed tomography diagnosis of cardiovascular involvement in behcet syndrome

    International Nuclear Information System (INIS)

    Zhi Aihua; Dai Ruping; Jiang Shiliang; Lu Bin; Zhang Pei

    2009-01-01

    Objective: To evaluate the computed tomography (CT) characteristics of cardiovascular involvement in Behcet syndrome. Methods: Eleven patients with clinically diagnosed Behcet syndrome were studied retrospectively from July 1995 to December 2007. Electron beam CT or 64-slice helical CT scanner was used and CT characteristics were reviewed. Results: Eleven patients were diagnosed according to the criteria reported by the international study group for Behcet syndrome. Of them, 4 patients presented with aortic valve prolapse (2 patients with mitral valve prolapse), false aneurysm of right coronary artery was demonstrated in 2 patients, false aneurysm of left subclavian artery, aortic aneurysm and penetrating ulcers, aortic arch false aneurysm, aortic dissection, pulmonary embolism and interatrial septum aneurysm in 1 case, respectively. Conclusion: CT is a very useful method for the diagnosis and follow-up of Behcet syndrome. (authors)

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

    Science.gov (United States)

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

    2013-12-01

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

  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. The non-syndromic familial thoracic aortic aneurysms and dissections maps to 15q21 locus

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    Chandok Gurangad

    2010-10-01

    Full Text Available Abstract Background Thoracic aortic aneurysms and dissections (TAAD is a critical condition that often goes undiagnosed with fatal consequences. While majority of the cases are sporadic, more than 20% are inherited as a single gene disorder. The most common familial TAA is Marfan syndrome (MFS, which is primarily caused by mutations in fibrillin-1 (FBN1 gene. Patients with FBN1 mutations are at higher risk for dissection compared to other patients with similar size aneurysms. Methods Fifteen family members were genotyped using Affymetrix-10K genechips. A genome-wide association study was carried out using an autosomal dominant model of inheritance with incomplete penetrance. Mutation screening of all exons and exon-intron boundaries of FBN1 gene which reside near the peak Lod score was carried out by direct sequencing. Results The index case presented with agonizing substernal pain and was found to have TAAD by transthoracic echocardiogram. The family history was significant for 3 first degree relatives with TAA. Nine additional family members were diagnosed with TAA by echocardiography examinations. The affected individuals had no syndromic features. A genome-wide analysis of linkage mapped the disease gene to a single locus on chromosome 15q21 with a peak Lod score of 3.6 at fibrillin-1 (FBN1 gene locus (odds ratio > 4000:1 in favour of linkage, strongly suggesting that FBN1 is the causative gene. No mutation was identified within the exons and exon-intron boundaries of FBN1 gene that segregated with the disease. Haplotype analysis identified additional mutation carriers who had previously unknown status due to borderline dilation of the ascending aorta. Conclusions A familial non-syndromic TAAD is strongly associated with the FBN1 gene locus and has a malignant disease course often seen in MFS patients. This finding indicates the importance of obtaining detailed family history and echocardiographic screening of extended relatives of patients

  18. Candy-Plug Technique Using an Excluder Aortic Extender for Distal Occlusion of a Large False Lumen Aneurysm in Chronic Aortic Dissection.

    Science.gov (United States)

    Ogawa, Yukihisa; Nishimaki, Hiroshi; Chiba, Kiyoshi; Murakami, Kenji; Sakurai, Yuka; Fujiwara, Keishi; Miyairi, Takeshi; Nakajima, Yasuo

    2016-06-01

    To describe the candy-plug technique using an Excluder aortic extender for distal occlusion of a large false lumen aneurysm in chronic aortic dissection. A 60-year-old female patient with a history of chronic type B aortic dissection and high-dose steroid use for Churg-Strauss syndrome developed a large 6.2 cm maximum diameter false lumen aneurysm. She underwent thoracic endovascular aortic repair from the left common carotid artery to the descending aorta to cover the proximal entry at the level of distal arch, with coil embolization of the left subclavian artery. To occlude the large false lumen from the reentry just below the level of the left renal artery ostium, a modified 32×45-mm Excluder aortic extender was deployed in the false lumen through the reentry, and a 16-mm Amplatzer Vascular Plug I was deployed in the waist of the modified Excluder aortic extender for complete occlusion. No obvious technical complication was seen. Contrast-enhanced computed tomography at 1 and 14 months revealed no endoleaks and showed complete false lumen thrombosis. The candy-plug technique using the Excluder aortic extender is feasible for occlusion of a large false lumen aneurysm in chronic aortic dissection. © The Author(s) 2016.

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

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    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. Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy.

    Science.gov (United States)

    Lacro, Ronald V; Guey, Lin T; Dietz, Harry C; Pearson, Gail D; Yetman, Anji T; Gelb, Bruce D; Loeys, Bart L; Benson, D Woodrow; Bradley, Timothy J; De Backer, Julie; Forbus, Geoffrey A; Klein, Gloria L; Lai, Wyman W; Levine, Jami C; Lewin, Mark B; Markham, Larry W; Paridon, Stephen M; Pierpont, Mary Ella; Radojewski, Elizabeth; Selamet Tierney, Elif Seda; Sharkey, Angela M; Wechsler, Stephanie Burns; Mahony, Lynn

    2013-05-01

    The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects. Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area-adjusted aortic root diameter z-score >3.0. The mean age at study entry was 11.2 years, 60% were male, and 25% were older teenagers and young adults. The median aortic root diameter z-score was 4.0. Aortic root diameter z-score did not vary with age. Mitral valve prolapse and mitral regurgitation were more common in females. Among those with a positive family history, 56% had a family member with aortic surgery, and 32% had a family member with a history of aortic dissection. Baseline demographic, clinical, and anthropometric characteristics of the randomized cohort are representative of patients in this population with moderate to severe aortic root dilation. The high percentage of young subjects with relatives who have had aortic dissection or surgery illustrates the need for more definitive therapy; we expect that the results of the study and the wealth of systematic data collected will make an important contribution to the management of individuals with Marfan syndrome. Copyright © 2013 Mosby, Inc. All rights reserved.

  1. Redox stress in Marfan syndrome: Dissecting the role of the NADPH oxidase NOX4 in aortic aneurysm.

    Science.gov (United States)

    Jiménez-Altayó, Francesc; Meirelles, Thayna; Crosas-Molist, Eva; Sorolla, M Alba; Del Blanco, Darya Gorbenko; López-Luque, Judit; Mas-Stachurska, Aleksandra; Siegert, Ana-Maria; Bonorino, Fabio; Barberà, Laura; García, Carolina; Condom, Enric; Sitges, Marta; Rodríguez-Pascual, Fernando; Laurindo, Francisco; Schröder, Katrin; Ros, Joaquim; Fabregat, Isabel; Egea, Gustavo

    2018-04-01

    Marfan syndrome (MFS) is characterized by the formation of ascending aortic aneurysms resulting from altered assembly of extracellular matrix fibrillin-containing microfibrils and dysfunction of TGF-β signaling. Here we identify the molecular targets of redox stress in aortic aneurysms from MFS patients, and investigate the role of NOX4, whose expression is strongly induced by TGF-β, in aneurysm formation and progression in a murine model of MFS. Working models included aortae and cultured vascular smooth muscle cells (VSMC) from MFS patients, and a NOX4-deficient Marfan mouse model (Fbn1 C1039G/+ -Nox4 -/- ). Increased tyrosine nitration and reactive oxygen species levels were found in the tunica media of human aortic aneurysms and in cultured VSMC. Proteomic analysis identified nitrated and carbonylated proteins, which included smooth muscle α-actin (αSMA) and annexin A2. NOX4 immunostaining increased in the tunica media of human Marfan aorta and was transcriptionally overexpressed in VSMC. Fbn1 C1039G/+ -Nox4 -/- mice aortas showed a reduction of fragmented elastic fibers, which was accompanied by an amelioration in the Marfan-associated enlargement of the aortic root. Increase in the contractile phenotype marker calponin in the tunica media of MFS mice aortas was abrogated in Fbn1 C1039G/+ -Nox4 -/- mice. Endothelial dysfunction evaluated by myography in the Marfan ascending aorta was prevented by the absence of Nox4 or catalase-induced H 2 O 2 decomposition. We conclude that redox stress occurs in MFS, whose targets are actin-based cytoskeleton members and regulators of extracellular matrix homeostasis. Likewise, NOX4 have an impact in the progression of the aortic dilation in MFS and in the structural organization of the aortic tunica media, the VSMC phenotypic modulation, and endothelial function. Copyright © 2018 Elsevier Inc. All rights reserved.

  2. Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: a prospective, multicenter, comparative study.

    Science.gov (United States)

    Coselli, Joseph S; Volguina, Irina V; LeMaire, Scott A; Sundt, Thoralf M; Connolly, Heidi M; Stephens, Elizabeth H; Schaff, Hartzell V; Milewicz, Dianna M; Vricella, Luca A; Dietz, Harry C; Minard, Charles G; Miller, D Craig

    2014-06-01

    To compare the 1-year results after aortic valve-sparing (AVS) or valve-replacing (AVR) aortic root replacement from a prospective, international registry of 316 patients with Marfan syndrome (MFS). Patients underwent AVS (n = 239, 76%) or AVR (n = 77, 24%) aortic root replacement at 19 participating centers from 2005 to 2010. One-year follow-up data were complete for 312 patients (99%), with imaging findings available for 293 (94%). The time-to-events were compared between groups using Kaplan-Meier curves and Cox proportional hazards models. Two patients (0.6%)--1 in each group--died within 30 days. No significant differences were found in early major adverse valve-related events (MAVRE; P = .6). Two AVS patients required early reoperation for coronary artery complications. The 1-year survival rates were similar in the AVR (97%) and AVS (98%) groups; the procedure type was not significantly associated with any valve-related events. At 1 year and beyond, aortic regurgitation of at least moderate severity (≥2+) was present in 16 patients in the AVS group (7%) but in no patients in the AVR group (P = .02). One AVS patient required late AVR. AVS aortic root replacement was not associated with greater 30-day mortality or morbidity rates than AVR root replacement. At 1 year, no differences were found in survival, valve-related morbidity, or MAVRE between the AVS and AVR groups. Of concern, 7% of AVS patients developed grade ≥2+ aortic regurgitation, emphasizing the importance of 5 to 10 years of follow-up to learn the long-term durability of AVS versus AVR root replacement in patients with MFS. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  3. [DISORDERS OF LUNG FUNCTION IN THE EARLY POSTOPERATIVE PERIOD IN PATIENTS OPERATED ON THE ASCENDING AORTA WITH THE EXPANSION OF INTERVENTION ON THE AORTIC ARCH].

    Science.gov (United States)

    Bokeria, L A; Nikitin, E S; Mironenko, V A; Rychin, S V; Vyganovskaya, N B; Makrushin, I M; Gordeev, S L

    2015-01-01

    Precapillary artery--arterial (bronchial artery--pulmonay artery) anastomoses, come into play in cases where the locking artery open and alveoli washed arterialized blood. Normally, these anastomoses are closed. Intrapulmonary shunts carry blood supply acinar hinder extraordinary circumstances (chronic pulmonary embolism, surgery, occurring in cardiopulmonary bypass, when the flaw of blood through the Iungs is suspended for the duration of cardiopulmonary bypass). Patients operated on the ascending aorta with the expansion of intervention on the nortic arch during the correction of pathology carry blood supply only the central nervous system. All other organs and systems at this time were protected by mild hypothermia. However, during circulatory arrest, required for correction of pathology acinar damage as a result of hypoxia, probably because there is no blood flow in the pulmonary artery and there is no blood flow in the bronchial arteries. Last, in the ordinary course of artificial circulation, capable due to arterio-arterial pulmonary anastomoses prevent hypoxic damage to acinar, not lead to persistent hypoxemia in the immediate postoperative period in these patients, as a result of violations of lung diffusion capacity.

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

    International Nuclear Information System (INIS)

    Dutra, Bruno Landim; Campos, Lenilton da Costa; Marques, Helder de Castro; Vilela, Vagner Moyses; Duque, Andre Geraldo da Silva

    2015-01-01

    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)

  5. 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)

  6. A comparison of Selective Aortic Arch Perfusion and Resuscitative Endovascular Balloon Occlusion of the Aorta for the management of hemorrhage-induced traumatic cardiac arrest: A translational model in large swine.

    Directory of Open Access Journals (Sweden)

    Ed B G Barnard

    2017-07-01

    Full Text Available Survival rates remain low after hemorrhage-induced traumatic cardiac arrest (TCA. Noncompressible torso hemorrhage (NCTH is a major cause of potentially survivable trauma death. Resuscitative Endovascular Balloon Occlusion of the Aorta (REBOA at the thoracic aorta (Zone 1 can limit subdiaphragmatic blood loss and allow for IV fluid resuscitation when intrinsic cardiac activity is still present. Selective Aortic Arch Perfusion (SAAP combines thoracic aortic balloon hemorrhage control with intra-aortic oxygenated perfusion to achieve return of spontaneous circulation (ROSC when cardiac arrest has occurred.Male Yorkshire Landrace cross swine (80.0 ± 6.0 kg underwent anesthesia, instrumentation for monitoring, and splenectomy. TCA was induced by laparoscopic liver lobe resection combined with arterial catheter blood withdrawal to achieve a sustained systolic blood pressure <10 mmHg, cardiac arrest. After 3 min of arrest, swine were allocated to one of three interventions: (1 REBOA plus 4 units of IV fresh whole blood (FWB, (2 SAAP with oxygenated lactated Ringer's (LR, 1,600 mL/2 min, or (3 SAAP with oxygenated FWB 1,600 mL/2 min. Primary endpoint was survival to the end of 60 min of resuscitation, a simulated prehospital phase. Thirty animals were allocated to 3 groups (10 per group-5 protocol exclusions resulted in a total of 35 animals being used. Baseline measurements and time to cardiac arrest were not different amongst groups. ROSC was achieved in 0/10 (0%, 95% CI 0.00-30.9 REBOA, 6/10 (60%, 95% CI 26.2-87.8 SAAP-LR and 10/10 (100%, 95% CI 69.2-100.0 SAAP-FWB animals, p < 0.001. Survival to end of simulated 60-minute prehospital resuscitation was 0/10 (0%, 95% CI 0.00-30.9 for REBOA, 1/10 (10%, 95% CI 0.25-44.5 for SAAP-LR and 9/10 (90%, 95% CI 55.5-99.7 for SAAP-FWB, p < 0.001. Total FWB infusion volume was similar for REBOA (2,452 ± 0 mL and SAAP-FWB (2,250 ± 594 mL. This study was undertaken in laboratory conditions, and as such may have

  7. 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.

  8. Decision-making in aortic root surgery in Marfan syndrome: bleeding, thromboembolism and risk of reintervention after valve-sparing or mechanical aortic root replacement.

    Science.gov (United States)

    Schoenhoff, Florian S; Langhammer, Bettina; Wustmann, Kerstin; Reineke, David; Kadner, Alexander; Carrel, Thierry

    2015-12-01

    Valve-sparing root replacement (VSRR) is thought to reduce the rate of thromboembolic and bleeding events compared with aortic root replacement using a mechanical aortic root replacement (MRR) with a composite graft by avoiding oral anticoagulation. But as VSRR carries a certain risk for subsequent reinterventions, decision-making in the individual patient can be challenging. Of 100 Marfan syndrome (MFS) patients who underwent 169 aortic surgeries and were followed at our institution since 1995, 59 consecutive patients without a history of dissection or prior aortic surgery underwent elective VSRR or MRR and were retrospectively analysed. VSRR was performed in 29 (David n = 24, Yacoub n = 5) and MRR in 30 patients. The mean age was 33 ± 15 years. The mean follow-up after VSRR was 6.5 ± 4 years (180 patient-years) compared with 8.8 ± 9 years (274 patient-years) after MRR. Reoperation rates after root remodelling (Yacoub) were significantly higher than after the reimplantation (David) procedure (60 vs 4.2%, P = 0.01). The need for reinterventions after the reimplantation procedure (0.8% per patient-year) was not significantly higher than after MRR (P = 0.44) but follow-up after VSRR was significantly shorter (P = 0.03). There was neither significant morbidity nor mortality associated with root reoperations. There were no neurological events after VSRR compared with four stroke/intracranial bleeding events in the MRR group (log-rank, P = 0.11), translating into an event rate of 1.46% per patient-year following MRR. The calculated annual failure rate after VSRR using the reimplantation technique was lower than the annual risk for thromboembolic or bleeding events. Since the perioperative risk of reinterventions following VSRR is low, patients might benefit from VSRR even if redo surgery may become necessary during follow-up. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  9. Molecular genetic analysis of individuals with Williams syndrome and supravalvar aortic stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Smoot, L.B.; Lacro, R.V.; Kunkel, L.M. [Children`s Hospital, Boston, MA (United States); Pober, B. [Yale Univ., New Haven, CT (United States)

    1994-09-01

    Mutations at the elastin locus (chromosome 7q11.23) have been demonstrated in individuals with Williams syndrome (WS) and familial supravalvar aortic stenosis (SVAS). Relationships between elastin mutations and vascular and/or neurodevelopmental pathology have yet to be defined. In determining phenotype-genotype correlations in WS/SVAS, we examined 35 individuals with sporadic WS, families with SVAS affecting multiple members, and sporadic cases of isolated obstructive vascular disease. Full length elastin cDNA was used to probe a human genomic library from which multiple elastin genomic clones have been isolated and ordered relative to the elastin gene, covering a minimum of 35 kb. (Additional genomic clones are being obtained by {open_quote}walking{close_quote} 5{prime} and 3{prime} to elastin.) Elastin genomic clones were used as probes in fluorescent in situ hybridization of metaphase chromosomes from WS/SVAS patients. Preliminary analysis confirms elastin deletions in WS patients, but have not yet been demonstrated in patients with isolated vascular disease using this technique. Results of deletional analysis in individuals representing a wide spectrum of phenotypes will be presented.

  10. 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

  11. Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome.

    Science.gov (United States)

    Laraja, Kristin; Sadhwani, Anjali; Tworetzky, Wayne; Marshall, Audrey C; Gauvreau, Kimberlee; Freud, Lindsay; Hass, Cara; Dunbar-Masterson, Carolyn; Ware, Janice; Lafranchi, Terra; Wilkins-Haug, Louise; Newburger, Jane W

    2017-05-01

    To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment. Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory. Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar. Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Forbidden word entropy of cerebral oximetric values predicts postoperative neurocognitive decline in patients undergoing aortic arch surgery under deep hypothermic circulatory arrest

    Directory of Open Access Journals (Sweden)

    Anna Dubovoy

    2017-01-01

    Full Text Available Purpose: Up to 53% of cardiac surgery patients experience postoperative neurocognitive decline. Cerebral oximetry is designed to detect changes in cerebral tissue saturation and therefore may be useful to predict which patients are at risk of developing neurocognitive decline. Methods: This is a retrospective analysis of a prospective study originally designed to determine if treatment of cerebral oximetry desaturation is associated with improvement in postoperative cognitive dysfunction in patients undergoing aortic reconstruction under deep hypothermic circulatory arrest. Cognitive function was measured, preoperatively and 3 months postoperatively, with 15 neuropsychologic tests administered by a psychologist; the individual test scores were summed and normalized. Bilateral cerebral oximetry data were stored and analyzed using measures of entropy. Cognitive decline was defined as any decrease in the summed normalized score from baseline to 3 months. Results: Seven of 17 (41% patients suffered cognitive decline. There was no association between baseline cerebral oximetry and postoperative cognitive dysfunction. Nor were changes in oximetry values associated with cognitive decline. However, cognitive decline was associated with loss of forbidden word entropy (FwEn (correlation: Rho ρ = 0.51, P = 0.037 for left cerebral oximetry FwEn and ρ = 0.54, P = 0.025 for right cerebral oximetry FwEn. Conclusion: Postoperative cognitive decline was associated with loss of complexity of the time series as shown by a decrease in FwEn from beginning to end of the case. This suggests that regulation of cerebral oximetry is different between those who do and those who do not develop cognitive decline.

  13. Digital subtraction angiography in patients with Marfan's syndrome

    International Nuclear Information System (INIS)

    Rauber, K.; Riemann, H.

    1987-01-01

    Marfan's syndrome is a rare inborn error of metabolism. Marfan patients are prone to aneurysms of the ascending aorta and run a high risk of rupture of the aortic arch. The diameter of the aneurysm is the most important predictor of the risk and therefore the leading point for surgical interventions. IV and IA-DSA according to our experiences are simple and effective methods in pre- and postoperative evaluation of patients with the syndrome. (orig.) [de

  14. 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

  15. Enhanced caspase activity contributes to aortic wall remodeling and early aneurysm development in a murine model of Marfan syndrome.

    Science.gov (United States)

    Emrich, Fabian C; Okamura, Homare; Dalal, Alex R; Penov, Kiril; Merk, Denis R; Raaz, Uwe; Hennigs, Jan K; Chin, Jocelyn T; Miller, Miquell O; Pedroza, Albert J; Craig, Juliana K; Koyano, Tiffany K; Blankenberg, Francis G; Connolly, Andrew J; Mohr, Friedrich W; Alvira, Cristina M; Rabinovitch, Marlene; Fischbein, Michael P

    2015-01-01

    Rupture and dissection of aortic root aneurysms remain the leading causes of death in patients with the Marfan syndrome, a hereditary connective tissue disorder that affects 1 in 5000 individuals worldwide. In the present study, we use a Marfan mouse model (Fbn1(C1039G/+)) to investigate the biological importance of apoptosis during aneurysm development in Marfan syndrome. Using in vivo single-photon emission computed tomographic-imaging and ex vivo autoradiography for Tc99m-annexin, we discovered increased apoptosis in the Fbn1(C1039G/+) ascending aorta during early aneurysm development peaking at 4 weeks. Immunofluorescence colocalization studies identified smooth muscle cells (SMCs) as the apoptotic cell population. As biological proof of concept that early aortic wall apoptosis plays a role in aneurysm development in Marfan syndrome, Fbn1(C1039G/+) mice were treated daily from 2 to 6 weeks with either (1) a pan-caspase inhibitor, Q-VD-OPh (20 mg/kg), or (2) vehicle control intraperitoneally. Q-VD-OPh treatment led to a significant reduction in aneurysm size and decreased extracellular matrix degradation in the aortic wall compared with control mice. In vitro studies using Fbn1(C1039G/+) ascending SMCs showed that apoptotic SMCs have increased elastolytic potential compared with viable cells, mostly because of caspase activity. Moreover, in vitro (1) cell membrane isolation, (2) immunofluorescence staining, and (3) scanning electron microscopy studies illustrate that caspases are expressed on the exterior cell surface of apoptotic SMCs. Caspase inhibition attenuates aneurysm development in an Fbn1(C1039G/+) Marfan mouse model. Mechanistically, during apoptosis, caspases are expressed on the cell surface of SMCs and likely contribute to elastin degradation and aneurysm development in Marfan syndrome. © 2014 American Heart Association, Inc.

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

    DEFF Research Database (Denmark)

    Cleemann, Line; Mortensen, Kristian H; Holm, Kirsten

    2010-01-01

    subjects. The mean age of the patients was 17 +/- 3.3 years. Magnetic resonance imaging was performed for all the patients. The thoracic aortic diameters of the patients were measured at nine positions. Adjustment for body surface area (BSA) was performed. The outcome for the patients was measured in terms...... of absolute and BSA-adjusted aortic dilation. In TS, both the absolute and the BSA-adjusted mean aortic diameters were smaller than or comparable with those of the control subjects. However, individual aortic dilation at one to four positions was found in four TS patients according to the uncorrected data...... and in five TS patients after BSA-adjustment. The aortic diameters correlated with height, weight, body mass index (BMI), and BSA at all positions (R = 0.34-0.60; all p

  17. Management and Outcomes of Childhood Renal Artery Stenosis and Middle Aortic Syndrome.

    Science.gov (United States)

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

    2018-01-24

    Renal artery stenosis (RAS) in isolation or in conjunction with middle aortic syndrome (MAS), are important vascular causes of childhood hypertension. Few longitudinal studies have assessed the risk of surgical or endovascular intervention, and outcomes by etiology or extent of vascular disease. In a retrospective study of 93 children seen over 30 years with RAS and/or MAS, data on vascular involvement (isolated RAS vs RAS with MAS), etiology (unknown, inflammatory, or genetic), and management were collected. Time to first intervention (endovascular or surgical) was assessed by Cox regression. Mixed-effects analysis examined the longitudinal change in blood pressure after intervention compared to antihypertensive medications alone. Children were 7.0±5.4 years old. Etiology was unknown in 50%, genetic in 26% and inflammatory in 24% of children. Children had isolated RAS (49%) or MAS with or without RAS (51%). Overall, 70% were managed with surgical or endovascular intervention. After adjusting for age, sex, and systolic blood pressure, children with unknown etiology had a higher risk of intervention compared to those with genetic and inflammatory diseases (HR 3.1, 95% CI 1.7,5.6). Children with RAS and MAS were less likely to receive intervention (HR 0.4, 95% CI 0.2,0.8) than isolated RAS. Over a median follow-up of 2 years, 65% remained hypertensive. The longitudinal changes in systolic blood pressure did not differ by etiology, or between interventional and medical management. Hypertension persists despite endovascular or surgical management of childhood RAS and MAS highlighting the importance of close monitoring and ongoing medical management. © American Journal of Hypertension, Ltd 2018. All rights reserved. For Permissions, please email: journals.permissions@oup.com

  18. Cardiovascular Structure and Function in Children With Middle Aortic Syndrome and Renal Artery Stenosis.

    Science.gov (United States)

    Rumman, Rawan K; Slorach, Cameron; Hui, Wei; Matsuda-Abedini, Mina; Langlois, Valerie; Radhakrishnan, Seetha; Lorenzo, Armando J; Amaral, Joao; Mertens, Luc; Parekh, Rulan S

    2017-12-01

    Middle aortic syndrome (MAS) is a narrowing of the abdominal aorta, often in conjunction with renal artery stenosis (RAS). Structure and function of the cardiovascular system are not well understood. In a prospective cross-sectional study, 35 children with MAS or RAS or both (MAS/RAS) were compared with 140 age-, sex-, and body surface area-matched healthy children. Vascular assessment included carotid intima-media thickness and carotid distensibility using B-mode ultrasound and central and peripheral pulse wave velocities using applanation tonometry. Left ventricular structure and function were assessed by 2-dimensional and speckle-tracking echocardiography. Children with MAS or RAS were 12.5±3.0 years old at enrollment, and 50% were men. Carotid intima-media thickness (0.54±0.10 versus 0.44±0.05 mm; P children with disease compared with healthy children; however, after adjustment for systolic blood pressure z score, only carotid intima-media thickness remained significantly higher in the MAS/RAS group compared with the controls (β=0.07 [0.03, 0.10]). Peripheral pulse wave velocities and carotid distensibility were normal. Children with disease had significantly increased left ventricular mass and changes in diastolic function (lower E/a ratio and lower e' velocities). Systolic parameters, including ejection fraction, global longitudinal and circumferential strain, were similar to controls. Our findings demonstrate that children with MAS or RAS have evidence of carotid and left ventricular remodeling, without peripheral arterial involvement, which suggests a localized disease process. Left ventricular systolic function is preserved; however, subtle changes in diastolic function are observed. Carotid vessel changes are consistent with a 5- to 10-year aging, which underscores the importance of blood pressure control. © 2017 American Heart Association, Inc.

  19. Relationship of metabolic syndrome with incident aortic valve calcium and aortic valve calcium progression: the Multi-Ethnic Study of Atherosclerosis (MESA).

    Science.gov (United States)

    Katz, Ronit; Budoff, Matthew J; Takasu, Junichiro; Shavelle, David M; Bertoni, Alain; Blumenthal, Roger S; Ouyang, Pamela; Wong, Nathan D; O'Brien, Kevin D

    2009-04-01

    Metabolic syndrome (MetS) has been associated with increased prevalence of aortic valve calcium (AVC) and with increased progression of aortic stenosis. The purpose of this study was to determine whether MetS is associated with increased risks for the development of new ("incident") AVC or for progression of established AVC as assessed by CT. The relationships of MetS or its components as well as of diabetes to risks for incident AVC or AVC progression were studied among participants with CT scans performed at baseline and at either year 2 or year 3 examinations in the Multi-Ethnic Study of Atherosclerosis (MESA). Of 5,723 MESA participants meeting criteria for inclusion, 1,674 had MetS by Adult Treatment Panel III criteria, whereas 761 had diabetes. Among the 5,123 participants without baseline AVC, risks for incident AVC, adjusted for time between scans, age, sex, race/ethnicity, LDL cholesterol, lipid-lowering medications, and smoking, were increased significantly for MetS (odds ratio [OR] 1.67 [95% CI 1.21-2.31]) or diabetes (2.06 [1.39-3.06]). In addition, there was an increase in incident AVC risk with increasing number of MetS components. Similar results were found using the International Diabetes Federation MetS criteria. Among the 600 participants (10.5%) with baseline AVC, neither MetS nor diabetes was associated with AVC progression. In the MESA cohort, MetS was associated with a significant increase in incident ("new") AVC, raising the possibility that MetS may be a potential therapeutic target to prevent AVC development.

  20. Aortic valve-sparing operation after correction of heart displacement due to pectus excavatum using Nuss procedure in a Marfan syndrome patient.

    Science.gov (United States)

    Fukunaga, Naoto; Yuzaki, Mitsuru; Hamakawa, Hiroshi; Nasu, Michihiro; Takahashi, Yutaka; Okada, Yukikatsu

    2012-01-01

    Cardiovascular surgery in the setting of chest wall deformities is a clinical challenge. Pectus excavatum, for example, can cause heart displacement to the left thoracic cavity, following the poor operative field. This report highlights a case in which a successful aortic valve-sparing operation via conventional median sternotomy after correction of the heart displacement due to pectus excavatum using Nuss procedure in Marfan syndrome. This technique can be one surgical option in Marfan syndrome patients with pectus excavatum and thoracic aortic aneurysm under close follow up.

  1. Long-Term Results of Aortic Root Surgery in Marfan Syndrome Patients: A Single-Center Experience.

    Science.gov (United States)

    Nicolo, Francesco; Romeo, Francesco; Lio, Antonio; Bovio, Emanuele; Scafuri, Antonio; Bassano, Carlo; Polisca, Patrizio; Pellegrino, Antonio; Nardi, Paolo; Chiariello, Luigi; Ruvolo, Giovanni

    2017-07-01

    The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation. Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29). No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33). The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

  2. Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

    Science.gov (United States)

    Ritter, Alyssa; Atzinger, Carrie; Hays, Brandon; James, Jeanne; Shikany, Amy; Neilson, Derek; Martin, Lisa; Weaver, Kathryn Nicole

    2017-06-01

    Hypermobile Ehlers-Danlos syndrome (hEDS) is a common inherited connective tissue disorder characterized by joint hypermobility. The natural history of aortic root dilation (AoD), a potential complication of EDS, has not been well characterized in this population. We describe the natural history of aortic root size in a large cohort of patients with hEDS. A cohort of 325 patients with HEDS was identified at Cincinnati Children's Hospital Medical Center (CCHMC), including 163 patients from a previous study. Medical records were reviewed and each participant's height, weight, and aortic dimensions from up to four echocardiograms were documented. Aortic root z-scores were calculated using two established formulas based on age (Boston or Devereux). Overall prevalence of AoD and prevalence by age were calculated and longitudinal regression was performed. The prevalence of AoD with a z-score ≥ 2.0 was 14.2% (46/325) and with a z-score of ≥3.0 was 5.5% (18/325). No significant increases in z-score were seen over time for patients with multiple echocardiograms. Participants under the age of 15 years had an average decline of 0.1 standard deviations (SDs)/year. No significant change was found after 15 of age. Between the ages of 15 and 21 years, Boston z-scores were 0.96 higher than Devereux z-scores. The nearly 1 z-score unit difference between formulas indicates caution prior to diagnosing AoD in patients with hEDS. In light of the low prevalence and lack of progression of AoD, routine echocardiograms may not be warranted for pediatric patients with hEDS. © 2017 Wiley Periodicals, Inc.

  3. 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

  4. Compliance of the normal-sized aorta in adolescents with Marfan syndrome: comparison of MR measurements of aortic distensibility and pulse wave velocity

    International Nuclear Information System (INIS)

    Eichhorn, J.G.; Ruediger, H.J.; Gorenflo, M.; Khalil, M.; Ulmer, H.; Krissak, R.; Kauczor, H.U.; Ley, S.; Universitaetsklinik Heidelberg; Arnold, R.; Universitaetskinderklinik Freiburg; Boese, J.; Siemens AG, Medical Solutions, Forchheim; Krug, R.; Fink, C.

    2007-01-01

    Purpose: To compare the aortic compliance of the normal-sized aorta of adolescents with Marfan syndrome and healthy controls using MR measurements of the aortic distensibility and pulse wave velocity. Materials and Methods: Fourteen patients (median age: 15 [9-21] years) and 11 healthy subjects (23 [12-32] years) were examined at 1.5 T. The MR protocol included 2D steady-state free precession (SSFP)-CINE MRI of the aortic distensibility and PC-MRI of the pulse wave velocity. All measurements were positioned perpendicular to the descending aorta at the level of the diaphragm for assessing the changes in the aortic cross-sectional areas and additionally above and below this plane for assessing the pulse wave velocity. In addition contrast-enhanced 3D-MR angiography was performed in adolescents with Marfan syndrome to exclude morphologic changes and to prove normal-sized aorta. Results: Compared with control subjects, adolescents with Marfan syndrome had significantly decreased distensibility and significantly increased pulse wave velocity (χ 2 -test, p = 0.0002) using an age-related non-linear regression analysis. The related aortic compliance was significantly decreased (χ 2 -test, p = 0.0002). There was a good correlation between the two methods (r = 0.86). A low intraobserver variability was found for both methods (≤ 2 %). (orig.)

  5. Proteção cerebral no tratamento cirúrgico dos aneurismas do arco aórtico: estudo experimental em cães Cerebral protection to be used during aortic arch aneurysms resection

    Directory of Open Access Journals (Sweden)

    Henrique Murad

    1993-09-01

    Full Text Available Os autores realizaram estudo experimental comparativo entre dois métodos de proteção cerebral utilizados na abordagem cirúrgica dos aneurismas do arco aórtico, avaliando a sua eficácia. Os métodos comparados foram a hipotermia sistêmica profunda isolada (menor que 20ºC com pinçamento arterial braquiocefálico e a hipotermia sistêmica profunda associada à perfusão carotídea seletiva. Dois grupos de 15 cães cada foram submetidos, respectivamente, a hipotermia sistêmica profunda com pinçamento arterial braquiocefálico (GRUPO I e a hipotermia sistêmica profunda associada a perfusão seletiva da carótida direita (GRUPO II. Foram colhidas amostras seriadas de sangue para análise das alterações metabólicas de pH e PaCO2 que ocorreram no retorno venoso cerebral, aferidas na veia jugular interna, bem como as alterações histopatológicas encontradas com 45 min, 90 min e 135 min de cada procedimento. Os resultados demonstraram que, apesar de ambos os métodos de proteção cerebral serem eficazes por um período de 45 minutos, o método utilizado no GRUPO II mostrou ser superior em períodos de até 90 minutos. Em períodos de 135 minutos os métodos tiveram resultados semelhantes, não oferecendo proteção cerebral adequada.The authors proposition is to make an experimental study of two methods of cerebral protection to be used during aortic arch aneurysm resection. The methods to be evaluated were profound systemic hypothermia (under 20oC with great vessels occlusion and profound systemic hypothermia with selective right carotid artery perfusion. Two groups of 15 dogs each were submitted either to profound systemic hypothermia with great vessels occlusion (GROUP I or to profound systemic hypothermia with selective right carotid artery perfusion (GROUP II. Serial jugular vein samples for pH and PaC02 were analyzed to evaluate ischemic cerebral metabolic derangements. Hystopathological studies were also made at 45, 90 and 135

  6. A quarter of a century of experience with aortic valve-sparing operations.

    Science.gov (United States)

    David, Tirone E; Feindel, Christopher M; David, Carolyn M; Manlhiot, Cedric

    2014-09-01

    To examine the late outcomes of aortic valve-sparing operations to treat patients with aortic root aneurysm with and without aortic insufficiency (AI) in a cohort of patients followed up prospectively since 1988. A total of 371 consecutive patients had undergone aortic valve-sparing surgery (mean age, 47 ± 15 years; 78% men) from 1988 through 2010. In addition to the aortic root aneurysm, 47% had moderate or severe AI, 35.5% had Marfan syndrome, 12.1% had type A aortic dissection, 9.2% had bicuspid aortic valve, 8.4% had mitral insufficiency, 16.1% had aortic arch aneurysm, and 10.2% had coronary artery disease. Reimplantation of the aortic valve was used in 296 patients and remodeling of the aortic root in 75. Cusp repair by plication of the free margin along the nodule of Arantius was used in 36.6% of patients, and reinforcement of the free margin with a double layer of fine Gore-Tex suture in 24.2%. The patients were followed up prospectively with images of the aortic root for a median follow-up of 8.9 ± 5.2 years. A total of 4 operative and 39 late deaths occurred. Survival at 18 years was 76.8% ± 4.31%, lower than that for the general population matched for age and gender. Age, type A aortic dissection, impaired ventricular function, and preoperative AI were associated with increased mortality on multivariable analysis. Reoperations on the aortic valve were performed in 8 patients for recurrent AI and in 2 for infective endocarditis. Freedom from reoperation on the aortic valve at 18 years was 94.8% ± 2.0%. No predictors of the need for reoperation were found on multivariable analysis. Eighteen patients developed AI greater than mild. Freedom from AI greater than mild at 18 years was 78.0% ± 4.8%. No predictors of recurrent AI were identified on multivariable analysis. Aortic valve-sparing operations continue to provide excellent clinical outcomes, although a slow but progressive deterioration of aortic valve function seems to occur during the first 2

  7. Digital subtraction angiography in patients with Marfan's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Rauber, K.; Riemann, H.

    1987-06-01

    Marfan's syndrome is a rare inborn error of metabolism. Marfan patients are prone to aneurysms of the ascending aorta and run a high risk of rupture of the aortic arch. The diameter of the aneurysm is the most important predictor of the risk and therefore the leading point for surgical interventions. IV and IA-DSA according to our experiences are simple and effective methods in pre- and postoperative evaluation of patients with the syndrome.

  8. Valve-sparing aortic root replacement†.

    Science.gov (United States)

    Koolbergen, David R; Manshanden, Johan S J; Bouma, Berto J; Blom, Nico A; Mulder, Barbara J M; de Mol, Bas A J M; Hazekamp, Mark G

    2015-02-01

    To evaluate our results of valve-sparing aortic root replacement and associated (multiple) valve repair. From September 2003 to September 2013, 97 patients had valve-sparing aortic root replacement procedures. Patient records and preoperative, postoperative and recent echocardiograms were reviewed. Median age was 40.3 (range: 13.4-68.6) years and 67 (69.1%) were male. Seven (7.2%) patients were younger than 18 years, the youngest being 13.4 years. Fifty-four (55.7%) had Marfan syndrome, 2 (2.1%) other fibrous tissue diseases, 15 (15.5%) bicuspid aortic valve and 3 (3.1%) had earlier Fallot repair. The reimplantation technique was used in all, with a straight vascular prosthesis in 11 (26-34 mm) and the Valsalva prosthesis in 86 (26-32 mm). Concomitant aortic valve repair was performed in 43 (44.3%), mitral valve repair in 10 (10.3%), tricuspid valve repair in 5 (5.2%) and aortic arch replacement in 3 (3.1%). Mean follow-up was 4.2 ± 2.4 years. Follow-up was complete in all. One 14-year old patient died 1.3 years post-surgery presumably of ventricular arrhythmia. One patient underwent reoperation for aneurysm of the proximal right coronary artery after 4.9 years and 4 patients required aortic valve replacement, 3 of which because of endocarditis after 0.1, 0.8 and 1.3 years and 1 because of cusp prolapse after 3.8 years. No thrombo-embolic complications occurred. Mortality, root reoperation and aortic regurgitation were absent in 88.0 ± 0.5% at 5-year follow-up. Results of valve-sparing root replacement are good, even in association with a high incidence of concomitant valve repair. Valve-sparing aortic root replacement can be performed at a very young age as long as an adult size prosthesis can be implanted. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  9. Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy

    NARCIS (Netherlands)

    Lacro, R.V.; Guey, L.T.; Dietz, H.C.; Pearson, G.D.; Yetman, A.T.; Gelb, B.D.; Loeys, B.L.; Benson, D.W.; Bradley, T.J.; Backer, J. de; Forbus, G.A.; Klein, G.L.; Lai, W.W.; Levine, J.C.; Lewin, M.B.; Markham, L.W.; Paridon, S.M.; Pierpont, M.E.; Radojewski, E.; Selamet Tierney, E.S.; Sharkey, A.M.; Wechsler, S.B.; Mahony, L.; et al.,

    2013-01-01

    BACKGROUND: The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and

  10. FLEETING MONOPARESIS AND AORTIC ARCH ATHEROMA ...

    African Journals Online (AJOL)

    A 72-year-old female with a history of hypertension and hyperlipidemia presented with alternating numbness, tingling sensations and weakness of both upper extremities with left shoulder pain. Each episode of ... The patient treated with aspirin, heparin and placed on warfarin for one year at target I.N.R of 2.0-3.0. She was ...

  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. PHACE(S) syndrome: Report of a case with new ocular and systemic manifestations.

    Science.gov (United States)

    Assari, Raheleh; Ziaee, Vahid; Moghimi, Sasan; Akbari, Mohammad Reza; Mirmohammadsadeghi, Arash

    2017-06-01

    To describe an infant with PHACE(S) syndrome [posterior fossa anomalies (P), hemangiomas (H), arterial anomalies (A), cardiac abnormalities and coarctation of aorta (C), eye abnormalities (E), and the sternal defects (S)] with unusual strabismus, congenital glaucoma, and new systemic manifestations. A 6-month-old girl was referred with large hemangiomas on the left side of the face. In the ocular examination, right esotropia and hypotropia, and limitation of elevation in adduction in the right eye were seen. Morning glory disk anomaly was seen in the left fundus. Intraocular pressure (IOP) was 28 mmHg in the right eye and 15 mmHg in the left eye. Brain computed tomography (CT) scan demonstrated Dandy-Walker malformation. In the CT angiography of the thoracic arteries, coarctation of aorta in descending part, the aberrant origin of the left subclavian artery from the end of the aortic arch, and anomalous origin of the left vertebral artery from the posterior aspect of the aortic arch were found. Therefore, the presence of large facial hemangioma, posterior fossa anomaly, aortic arch anomalies, and morning glory disk confirmed the diagnosis of PHACE(S) syndrome. Propranolol (0.5 mg/kg/day) was initiated to treat hemangioma and coarctation of aorta. Due to uncontrolled glaucoma, goniotomy was performed in the right eye 3 months after the first visit. One year after the initial visit, the hypotropia and esotropia of the right eye considerably decreased. To our knowledge, this report was the first report of a pattern like Brown's syndrome (may be called apparent Brown's syndrome) and the second report of the congenital glaucoma in a case of PHACE(S) syndrome. In addition, the anomalous origin of the vertebral artery from the aortic arch has not been reported in the PHACE(S) syndrome. Thus, the clinicians should perform the glaucoma work-up for each patient with this syndrome.

  13. Beals Syndrome

    Science.gov (United States)

    ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

  14. Valve-sparing aortic root reconstruction in children, teenagers, and young adults.

    Science.gov (United States)

    Tweddell, James S; Earing, Michael G; Bartz, Peter J; Dunham-Ingles, Jennifer L; Woods, Ronald K; Mitchell, Michael E

    2012-08-01

    We reviewed our experience with valve-sparing aortic root reconstruction (VSARR) using the sinus of Valsalva graft in children, teenagers, and young adults with connective tissue disorders. Results of a single-center experience with VSARR in children, teenagers, and young adults were retrospectively analyzed. End points were death, freedom from reintervention, and freedom from valve dysfunction. Between 2003 and 2010, 16 patients (Marfan, 9; Loeys Dietz syndrome, 6; conotruncal, 1) underwent VSARR. The mean age was 20±7.4 (range, 9 to 36 years). Indications for VSAAR were aortic root enlargement in 14 (sinus of Valsalva Z-score, 6.2±2) and aortic dissection in 2. Additional procedures included replacement of the ascending aorta in 7, with additional replacement of the aortic arch in 2. No early or late deaths occurred. One patient required a pacemaker. One patient with Loeys-Dietz syndrome required reoperation for aneurysmal dilatation of the coronary buttons. Two patients underwent replacement of the thoracoabdominal aorta for chronic dissection. Follow-up by echocardiography or magnetic resonance imaging at a mean of 33±29 months showed more than mild aortic regurgitation in 2 patients. Both patients with moderate aortic insufficiency also had a bicuspid aortic valve. VSARR using the sinus of Valsalva graft is a reproducible technique that achieves acceptable early and intermediate results. It is suitable for children, teenagers, and young adults. Anticoagulation is avoided. The procedure is appropriate for emergency operations but should be used with caution in patients with a bicuspid aortic valve. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  15. Increased prevalence of bicuspid aortic valve in Turner syndrome links with karyotype: the crucial importance of detailed cardiovascular screening.

    Science.gov (United States)

    Klásková, Eva; Zapletalová, Jiřina; Kaprálová, Sabina; Šnajderová, Marta; Lebl, Jan; Tüdös, Zbyněk; Pavlíček, Jan; Černá, Jana; Mihál, Vladimír; Stará, Veronika; Procházka, Martin

    2017-03-01

    Bicuspid aortic valve (BAV) represents one of the strongest risk factors for aortic dissection in Turner syndrome (TS). An exact relation between the occurrence of BAV and a particular karyotype has not been established yet. The aim of this study was to determine the association between karyotype and prevalence of BAV. Sixty-seven TS patients aged between 6.6 and 32.5 years underwent cardiac magnetic resonance imaging (MRI) study. They were divided into four cytogenetic subgroups-45,X karyotype (n=27); 45,X/46,XX mosaicism (n=17); structural abnormalities of the X chromosome (n=10); and 45,X/structural abnormality of the X chromosome mosaicism (n=13). Prevalence of BAV and odds ratio (OR) compared with the general population in the whole study group, and statistical comparison of prevalences of BAV among the individual subgroups were determined. Prevalence of BAV in the whole study group was established as 28.4% [OR 208.3 (95% CI - 103.8-418.0); p-valuekaryotype had the highest prevalence of BAV - 40.7%, p-valuekaryotype significantly predisposed to BAV (p-value=0.05). The 45,X karyotype is associated with the highest prevalence of BAV. Also, the presence of the 45,X cell line in any mosaic karyotype increases the probability of BAV.

  16. 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.

  17. Pregnancy and delivery in a patient with aortic prosthesis for Leriche syndrome

    OpenAIRE

    Sass,Nelson; Poli,Antonio Henrique Moura; Torloni,Maria Regina; Gibim,Paula Gabriela Monteagudo; Andrade,Januário de; Izukwa,Nilo Mitsuro

    2003-01-01

    CONTEXT: Leriche syndrome is a thrombotic obliteration of the bifurcation of the aorta, a rare condition that usually affects older men as a result of atherosclerosis. Women of childbearing age rarely need a vascular prosthesis (as a result of Leriche syndrome or other conditions) and there is no literature on an association between Leriche syndrome/vascular prosthesis and pregnancy/labor/delivery. CASE REPORT: A case of pregnancy and delivery in a 38-year-old patient with Leriche syndrome an...

  18. MR imaging of the thoracic aorta in patients with Marfan syndrome

    International Nuclear Information System (INIS)

    Sommerhoff, B.A.; Sechtem, U.P.; Schiller, N.B.; Higgins, C.B.

    1986-01-01

    Gated MR imaging was used to evaluate the thoracic aorta in 11 with Marfan syndrome, eight patients with aneurysm of the ascending aorta, and 20 healthy subjects. The aortic diameter was measured on transverse and sagittal images at the levels of the sinuses of Valsalva, the caudal portion of the ascending aorta, the prearch region, the middle arch, and the descending aorta. The sinus of Valsalva-prearch region aortic diameter ratio in patients with Marfan syndrome was significantly greater than in the two other groups, indicating the characteristic shape of the Maranoid aorta. MR imaging allows definitive measurements' of aortic dimensions and is a valuable noninvasive method for monitoring the course of aortic enlargement

  19. Inactivation of Bmp4 from the Tbx1 Expression Domain Causes Abnormal Pharyngeal Arch Artery and Cardiac Outflow Tract Remodeling

    Science.gov (United States)

    Nie, Xuguang; Brown, Christopher B.; Wang, Qin; Jiao, Kai

    2011-01-01

    Maldevelopment of outflow tract and aortic arch arteries is among the most common forms of human congenital heart diseases. Both Bmp4 and Tbx1 are known to play critical roles during cardiovascular development. Expression of these two genes partially overlaps in pharyngeal arch areas in mouse embryos. In this study, we applied a conditional gene inactivation approach to test the hypothesis that Bmp4 expressed from the Tbx1 expression domain plays a critical role for normal development of outflow tract and pharyngeal arch arteries. We showed that inactivation of Bmp4 from Tbx1-expressing cells leads to the spectrum of deformities resembling the cardiovascular defects observed in human DiGeorge syndrome patients. Inactivation of Bmp4 from the Tbx1 expression domain did not cause patterning defects, but affected remodeling of outflow tract and pharyngeal arch arteries. Our further examination revealed that Bmp4 is required for normal recruitment/differentiation of smooth muscle cells surrounding the PAA4 and survival of outflow tract cushion mesenchymal cells. PMID:21123999

  20. 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.

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

    Science.gov (United States)

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

    2008-08-01

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

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

    DEFF Research Database (Denmark)

    Leth, Peter Mygind; Knudsen, Peter Juel Thiis

    2015-01-01

    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...

  3. Cardiovascular Malformations in CHARGE Syndrome with DiGeorge Phenotype: Two Case Reports

    Directory of Open Access Journals (Sweden)

    Kazushi Yasuda

    2016-01-01

    Full Text Available Both CHARGE syndrome and DiGeorge anomaly are frequently accompanied by cardiovascular malformations. Some specific cardiovascular malformations such as interrupted aortic arch type B and truncus arteriosus are frequently associated with 22q11.2 deletion syndrome, while conotruncal defects and atrioventricular septal defects are overrepresented in patients with CHARGE syndrome. CHD7 gene mutation is identified in approximately two-thirds of patients with CHARGE syndrome, and chromosomal microdeletion at 22q11.2 is found in more than 95% of patients with 22q11.2 deletion syndrome. CHARGE syndrome is occasionally accompanied by DiGeorge phenotype. We report two patients with dysmorphic features of both CHARGE syndrome and 22q11.2 deletion syndrome. Although both of the two cases did not have 22q11.2 deletion, they had typical dysmorphic features of 22q11.2 deletion syndrome including cardiovascular malformations such as interrupted aortic arch type B. They also had characteristic features of CHARGE syndrome including ear malformation, genital hypoplasia, limb malformation, and endocrinological disorders. CHD7 gene mutation was confirmed in one of the two cases. When a patient with cardiovascular malformations frequently associated with 22q11.2 deletion syndrome does not have 22q11.2 deletion, we suggest that associated malformations characteristic of CHARGE syndrome should be searched for.

  4. Comparison of cine-MRI and transthoracic echocardiography for the assessment of aortic root diameters in patients with suspected Marfan syndrome

    International Nuclear Information System (INIS)

    Bannas, P.; Derlin, T.; Yamamura, J.; Lund, G.; Adam, G.; Rybczynski, M.; Sheikhzadeh, S.; Kodolitsch, Y. von; Groth, M.

    2015-01-01

    Patients with Marfan syndrome require repeated imaging for monitoring of aortic root aneurysms. Therefore, we evaluated the agreement and reproducibility of cine-MRI and echocardiography measurements of the sinuses of Valsalva in patients with suspected Marfan syndrome. 51 consecutive patients with suspected Marfan syndrome were prospectively examined using cine-MRI and echocardiography. Two readers independently measured aortic root diameters at the level of the sinuses of Valsalva in both cine-MRI and echocardiography. Statistics included intraclass correlation coefficient, Pearson correlation coefficient, Bland-Altman analysis, and two-sided t-test. In 38 of the 51 individuals (74.5 %), the diagnosis of Marfan syndrome was established according to the criteria of the Ghent-2 nosology. Cine-MRI measurements of the sinuses of Valsalva revealed a strong correlation with echocardiography (r=0.929), but a statistically significant bias of -1.0 mm (p<0.001). The mean absolute diameter for sinuses of Valsalva obtained by cine-MRI was 32.3 ± 5.8 mm as compared to 33.4 ± 5.4 mm obtained by echocardiography. Interobserver agreement of measurements of the sinuses of Valsalva was higher for cine-MRI than for echocardiography (p=0.029). Despite small, but statistically significant differences in terms of agreement and reproducibility, cine-MRI and echocardiographic measurements of aortic root diameters provide comparable results without a significant clinical difference. Therefore both techniques may be used for monitoring of the aortic root in patients with Marfan syndrome.

  5. 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.

  6. Traumatic Aortic Injury

    Directory of Open Access Journals (Sweden)

    Brianna Miner

    2016-09-01

    Full Text Available History of present illness: A 48-year-old male with unknown past medical history presents as a trauma after being hit by a car traveling approximately 25 miles per hour. On initial presentation, the patient is confused, combative, and not answering questions appropriately. The patient is hypotensive with a blood pressure of 68/40 and a heart rate of 50 beats per minute, with oxygen saturation at 96% on room air. FAST scan is positive for fluid in Morrison’s pouch, splenorenal space, and pericardial space. Significant findings: The initial chest x-ray showed an abnormal superior mediastinal contour (blue line, suggestive of a possible aortic injury. The CT angiogram showed extensive circumferential irregularity and outpouching of the distal aortic arch (red arrows compatible with aortic transection. In addition, there was a circumferential intramural hematoma, which extended through the descending aorta to the proximal infrarenal abdominal aorta (green arrow. There was also an extensive surrounding mediastinal hematoma extending around the descending aorta and supraaortic branches (purple arrows. Discussion: Traumatic aortic injury is a life-threatening event. The incidence of blunt thoracic aortic injury is low, between 1 to 2 percent of those patients with blunt thoracic trauma.1 However, approximately 80% of patients with traumatic aortic injury die at the scene.2 Therefore it is imperative to diagnose traumatic aortic injury in a timely fashion. The diagnosis can be difficult due to the non-specific signs and symptoms and other distracting injuries. Clinical suspicion should be based on the mechanism of the injury and the hemodynamic status of the patient. In any patient with blunt or penetrating trauma to the chest that is hemodynamically unstable, traumatic aortic injury should be on the differential. Chest x-ray can be used as a screening tool. A normal chest x-ray has a negative predictive value of approximately 97%. CTA chest is the

  7. [Stanford type B aortic dissection associated with coarctation of the aorta].

    Science.gov (United States)

    Senaha, Shigenobu; Uezu, Toru; Shimoji, Mitsuyoshi; Akasaki, Mitsuru

    2015-03-01

    We report a rare case of type B aortic dissection associated with coarctation of the aorta. A 35-year-old man had sudden dyspnea and severe back pain. Computed tomography revealed aortic coarctation at the distal aortic arch, and aortic dissection below the coarctation. The diameter of proximal descending aorta was enlarged to 52 mm. We electively performed excision of aortic coarctation and descending aortic graft replacement. Coarctation of the aorta has a poor prognosis, The risk of aortic rupture due to aortic dissection is very high, and the histological abnormality is also pointed out. Therefore we should perform aggressive surgical treatment.

  8. Técnica de perfusión selectiva cerebral vía subclavia para la corrección de patologías del arco aórtico Selective cerebral perfusion technique by subclavian approach for correction of aortic arch pathology

    Directory of Open Access Journals (Sweden)

    Alejandro Escobar

    2007-08-01

    Full Text Available Objetivo: describir y evaluar la técnica de perfusión subclavia para protección cerebral selectiva con hipotermia moderada. Métodos: estudio descriptivo de 19 pacientes, a quienes se les practicó corrección de disección o aneurisma del arco aórtico mediante la utilización de esta técnica. Resultados: entre septiembre de 2002 y septiembre de 2005, se intervinieron 19 pacientes. El 68,4% eran hombres, con un promedio de edad de 54,05 ± 13,53 años. La disección de aorta tipo A correspondió al 57,8%; el 42,2% restante tenía aneurismas en alguna porción de la aorta con compromiso del cayado. El 73,7% tenía insuficiencia valvular aórtica. El 47,3% se encontró en clase funcional I, el 31,5% en clase II y el 21% en clase III. El 36,8% requirió revascularización coronaria. El tiempo promedio de perfusión cerebral selectiva fue de 28,95 ± 8,73 minutos; la perfusión sistémica fue de 163,31 ± 32,15 minutos, el pinzamiento aórtico fue de 135,36 ± 34,48 minutos y la temperatura promedio fue de 27º ± 0,94º centígrados. Hubo tres defunciones. No ocurrieron complicaciones neurológicas definitivas. Conclusión: esta técnica puede ser estandarizada para cirugías electivas o emergentes. Es una técnica simple, reproducible, que permite períodos de tiempo más prolongados para la reconstrucción del arco aórtico sin producir isquemia del tejido cerebral, tiempos de circulación extracorpórea más cortos, pocas complicaciones por sangrado, disminución del riesgo de embolización cerebral anterógrada y un excelente resultado neurológico final.Objective: describe and evaluate the subclavian perfusion technique for selective cerebral protection with moderate hypothermia. Methods: descriptive study of 19 patients to whom correction of the dissection or aneurysm of the aortic arch through the utilization of this technique was practiced. Results: between September 2002 and September 2005, 19 patients were operated. 68.4% were men

  9. Aortic dissection

    Science.gov (United States)

    ... made in the chest or abdomen. Endovascular aortic repair. This surgery is done without any major surgical ... needed. If the heart arteries are involved, a coronary bypass is also performed. Outlook ... aneurysm - dissecting; Chest pain - aortic dissection; Thoracic aortic aneurysm - ...

  10. The thoracic aortography by Gd-DTPA enhanced ultrafast cine MR imaging. Assessment of thoracic aortic dilatation in aging and in patients with hypertension and aortic valve disease

    International Nuclear Information System (INIS)

    Matsumura, Kentaro; Nakase, Emiko; Kawai, Ichiyoshi; Saito, Takayuki; Kikkawa, Nobutada; Haiyama, Toru

    1995-01-01

    To assess the morphology of thoracic aorta, we had a trial of Gd-DTPA enhanced ultrafast cine MR imaging on the thoracic aorta. This method was provided with high quality thoracic aortogram during 15-20 seconds. In patients without hypertension and aortic valve disease, dimensions of ascending aorta and aortic arch were significantly correlated with aging. In patients with hypertension, dimensions of ascending aorta and aortic arch were significantly dilated. In patients with aortic valve disease, thoracic aorta was diffusely enlarged, especially in ascending aorta. Gd-DTPA enhanced ultrafact cine MR imaging was useful to assess the thoracic aortic anatomy and diseases. (author)

  11. Treatment of a Chronic Aneurysmal Aortic Dissection in a Patient with Marfan Syndrome Using a Staged Hybrid Procedure and a Fenestrated Endograft

    International Nuclear Information System (INIS)

    Walkden, R. Miles; Morgan, Rob A.; Loftus, Ian; Thompson, Matt

    2008-01-01

    Patients with aneurysmal dissections involving both the thoracic and the abdominal aorta are particularly challenging to treat with endovascular techniques because of the natural communications at the level of the visceral arteries. We present the case of a patient with Marfan syndrome with an aneurysmal aortic dissection involving the thoracic and abdominal aorta who was treated by a combination of endografts, surgical bypass, and a fenestrated tube graft.

  12. [Surgical treatment of cardiovascular manifestations of Marfan syndrome].

    Science.gov (United States)

    Fukada, J; Morishita, K; Kawaharada, N; Yamada, A; Baba, T; Harada, N; Abe, T

    2002-07-01

    The present study determines the effect of surgical treatment of cardiovascular manifestations of Marfan syndrome in 72 patients by 114 operations, during 34-year period. This therapy resulted in aortic root repair, aortic arch replacement, or both in 78, mitral valve repair in 9, descending thoracic aortic replacement in 14, thoracoabdominal aortic replacement in 10, and abdominal aortic replacement in 6, including total aortic replacement in 4 and nearly total aortic replacement in 4 patients. Fusiform aneurysms were present in the the ascending aorta in 37, the aortic arch in 2, the thoracoabdominal aorta in 2, and the abdominal aorta in 6 patients. Aortic dissection occurred in 40 (55.6%), including type A aortic dissection in 29 patients. Aortic root repair included separate valve-graft in 8, Bentall composite valve-graft in 25, composite valve-graft with button technique in 26, composite valve-graft with interposition graft technique in 10, and valve sparing procedure in 5 patients. The overall early (30-day) mortality was 7.9%. The early survival was 75% in separate valve-graft procedure and 99.2% in composite valve-graft procedure. Late coronary dehiscence did not occur in the patients with Bentall technique in which the reattachments of coronary ostia were performed in 2 layers, but occurred in 50% of patients with the coronary anastomoses in 1 layer. Aortic valve regurgitation relapsed in 2 of the 5 patients with valve sparing procedure. Event free rate for the patients with composite valve-graft using button technique was 81.1% at 10 years. There were 14 late deaths; dissection or rupture of the residual aorta, composite graft endocarditis and cardiac failure were the principle causes of late deaths. In conclusion, Marfan patients with cardiovascular diseases can undergo surgical treatment with a low operative risk and low morbidity. Although late endocarditis remains a serious problem, we believe that Marfan syndrome is a contraindication for valve

  13. Successful Repair of Type a Aortic Dissection in a Term Pregnancy: A Case Report

    Directory of Open Access Journals (Sweden)

    Kambiz Alizadeh

    2016-09-01

    Full Text Available Stanford type A acute aortic dissection (AAD is a very rare complication, with potentially lethal consequences in pregnancy. In fact, pregnancy has been regularly associated with the possibility of aortic. dissection in almost half of young women. Herein, we present the case of a 38-year-old woman in her 37th week of pregnancy. The patient’s medical history was indicative of G4L2Ab1(4 gestaitions,2 lives, 1 abortion. She developed persistent chronic pain in the neck, chest, and back, without nausea or vomiting while waking in the morning. The computed tomography angiogram was indicative of AAD. The medical decision was to perform a combination of cesarean section under general anesthesia and median sternotomy for the open aortic valve. The term newborn showed an Apgar score of 9-10. The coronary arteries were preserved and the valve was repaired at commissural positions. Dacron supracoronary tube graft was attached to the aortic anastomotic site and subsequently to the aortic arch. The patient was discharged on day three after surgery with a good general condition. Moreover, the results indicated that she and one of her brothers suffered from Marfan syndrome.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2002-01-01

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

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

    International Nuclear Information System (INIS)

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

    2002-01-01

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

  16. Comparison of Cine-MRI and Transthoracic Echocardiography for the Assessment of Aortic Root Diameters in Patients with Suspected Marfan Syndrome.

    Science.gov (United States)

    Bannas, P; Rybczynski, M; Sheikhzadeh, S; von Kodolitsch, Y; Derlin, T; Yamamura, J; Lund, G; Adam, G; Groth, M

    2015-11-01

    Patients with Marfan syndrome require repeated imaging for monitoring of aortic root aneurysms. Therefore, we evaluated the agreement and reproducibility of cine-MRI and echocardiography measurements of the sinuses of Valsalva in patients with suspected Marfan syndrome. 51 consecutive patients with suspected Marfan syndrome were prospectively examined using cine-MRI and echocardiography. Two readers independently measured aortic root diameters at the level of the sinuses of Valsalva in both cine-MRI and echocardiography. Statistics included intraclass correlation coefficient, Pearson correlation coefficient, Bland-Altman analysis, and two-sided t-test. In 38 of the 51 individuals (74.5 %), the diagnosis of Marfan syndrome was established according to the criteria of the Ghent-2 nosology. Cine-MRI measurements of the sinuses of Valsalva revealed a strong correlation with echocardiography (r = 0.929), but a statistically significant bias of -1.0  mm (p Marfan syndrome. © Georg Thieme Verlag KG Stuttgart · New York.

  17. Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection

    Directory of Open Access Journals (Sweden)

    R. K. Zhuraev

    2012-08-01

    Full Text Available The article presents the pathologic changes in the aortic wall in patients with MFS (three men at the age of 25, 27, 35 years operated on the aneurysm with dissection of the ascending aorta. Morphological changes in the middle of the shell of the aorta in patients with MFS were characterized by severe restructuring with profound irreversible alterative changes in all of its components. The pathological process captured media of the aorta throughout its entire length, not just in the areas of rupture and separation. The main pathomorphological signs of MFS were the focal accumulation of mucoid substances, ribbon-like nuclear-free zones, degenerative changes in smooth muscle cells, the formation of cystic media degeneration cavities, changes in elastic fibers - fragmentation, hyperelastosis, multiplication, thinning and straightening, areas of significant elastolysis. Multiple CMD was detected in all patients with MFS, the changes revealed the same type, but at different stages of the process.

  18. 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.

  19. 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.

  20. Can Brain Natriuretic Peptides and Osteoprotegerin Serve As Biochemical Markers for the Detection of Aortic Pathology in Children and Adolescents with Turner Syndrome?

    Directory of Open Access Journals (Sweden)

    Meenal Mavinkurve

    2017-07-01

    Full Text Available Turner syndrome (TS is a chromosomal disorder that affects 1:2,000 females. It results from either the complete or partial loss of the X chromosome as well as other aberrations. Clinical features of TS include short stature, delayed puberty, and congenital cardiac malformations. TS children also have an increased prevalence of cardiometabolic risk factors, which predisposes them to complications like coronary artery disease, cerebrovascular-related deaths, and aortic dissection. Early cardiac imaging, such as echocardiography and cardiac magnetic resonance imaging, are recommended to detect underlying aortic pathology. However, these modalities are limited by cost, accessibility, and are operator dependent. In view of these shortcomings, alternative methods, like vascular biomarkers, are currently being explored. There are only a few studies that have examined the relationship between B-type natriuretic peptide (BNP, N-terminal pro BNP (NT pro-BNP, and osteoprotegerin (OPG and aortic disease in TS, and thus the data are only in proof-of-concept stages. Further meticulous longitudinal studies are required before BNP, NT pro-BNP, and OPG are used as vascular biomarkers for the detection of aortic disease in childhood and adolescent TS.

  1. Cable strengthened arches

    NARCIS (Netherlands)

    Kamerling, M.W.

    2013-01-01

    The structural efficiency of arches, subjected to several variable loads, can be increased by strengthening these arches with cables. For these structures it can be necessary, especially in case the permanent load is small, to post-tension the cables to avoid any compression acting on the cables. A

  2. Diagnosis, imaging and clinical management of aortic coarctation

    NARCIS (Netherlands)

    Dijkema, Elles J; Leiner, Tim; Grotenhuis, Heynric B

    2017-01-01

    Coarctation of the aorta (CoA) is a well-known congenital heart disease (CHD), which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic

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

    NARCIS (Netherlands)

    Dingemans, Koert P.; Teeling, Peter; van der Wal, Allard C.; Becker, Anton E.

    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

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

    International Nuclear Information System (INIS)

    Shoji, Shuneki

    2003-01-01

    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

  5. 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

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

    OpenAIRE

    Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

    2014-01-01

    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 vent...

  7. Aortic root replacement after previous surgical intervention on the aortic valve, aortic root, or ascending aorta.

    Science.gov (United States)

    Kirsch, E W Matthias; Radu, N Costin; Mekontso-Dessap, Armand; Hillion, Marie-Line; Loisance, Daniel

    2006-03-01

    Aortic root replacement after a previous operation on the aortic valve, aortic root, or ascending aorta remains a major challenge. Records of 56 consecutive patients (44 men; mean age, 56.4 +/- 13.6 years) undergoing reoperative aortic root replacement between June 1994 and June 2005 were reviewed retrospectively. Reoperation was performed 9.4 +/- 6.7 years after the last cardiac operation. Indications for reoperation were true aneurysm (n = 14 [25%]), false aneurysm (n = 10 [18%]), dissection or redissection (n = 9 [16%]), structural or nonstructural valve dysfunction (n = 10 [18%]), prosthetic valve-graft infection (n = 12 [21%]), and miscellaneous (n = 1 [2%]). Procedures performed were aortic root replacement (n = 47 [84%]), aortic root replacement plus mitral valve procedure (n = 5 [9%]), and aortic root replacement plus arch replacement (n = 4 [7%]). In 14 (25%) patients coronary artery bypass grafting had to be performed unexpectedly during the same procedure or immediately after the procedure to re-establish coronary perfusion. Hospital mortality reached 17.9% (n = 10). Multivariate logistic regression analysis revealed the need for unplanned perioperative coronary artery bypass grafting as the sole independent risk factor for hospital death (P = .005). Actuarial survival was 83.8% +/- 4.9% at 1 month, 73.0% +/- 6.3% at 1 year, and 65.7% +/- 9.0% at 5 years after the operation. One patient had recurrence of endocarditis 6.7 months after the operation and required repeated homograft aortic root replacement. Reoperative aortic root replacement remains associated with a high postoperative mortality. The need to perform unplanned coronary artery bypass grafting during reoperative aortic root replacement is a major risk factor for hospital death. The optimal technique for coronary reconstruction in this setting remains to be debated.

  8. 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.

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

    Science.gov (United States)

    Nemani, Lalita; Barik, Ramachandra; Patnaik, Amar Narayana; Mishra, Ramesh C; Rao, Amaresh M; Kapur, Pragati

    2014-09-01

    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.

  10. Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: Analysis of early outcome.

    Science.gov (United States)

    Volguina, Irina V; Miller, D Craig; LeMaire, Scott A; Palmero, Laura C; Wang, Xing Li; Connolly, Heidi M; Sundt, Thoralf M; Bavaria, Joseph E; Dietz, Harry C; Milewicz, Dianna M; Coselli, Joseph S

    2009-05-01

    A prospective, international registry study was initiated to provide contemporary comparative data on short-term clinical outcomes after aortic valve-sparing and aortic valve-replacing root operations in patients with Marfan syndrome. The purpose of this initial report is to describe the study design and to compare early outcomes in the first 151 enrolled patients. We assessed 30-day outcomes in 151 patients who met strict Ghent diagnostic criteria for Marfan syndrome and underwent aortic root replacement with either valve-replacing (n = 46) or valve-sparing techniques (n = 105) at one of 18 participating centers. In the valve replacement group, a mechanical composite valve graft was used in 39 (85%) patients and a bioprosthetic valve in 7 (15%). In the valve-sparing group, David V procedures were performed in 57 (54%) patients, David I in 38 (36%), David IV in 8 (8%), Florida sleeve in 1 (1%), and Yacoub remodeling in 1 (1%). No in-hospital or 30-day deaths occurred. Despite longer crossclamp and cardiopulmonary bypass times in the valve-sparing group, there were no significant between-group differences in postoperative complications. Thirty-day valve-related complications occurred in 2 (4%) patients undergoing valve replacement and in 3 (3%) undergoing valve-sparing procedures (P = .6). The analysis of early outcomes revealed that valve-sparing techniques were the most common approach to root replacement in patients with Marfan syndrome in these centers. The complexity of valve-sparing root replacement did not translate into any demonstrable adverse early outcomes. Subsequent analysis will compare the 3-year durability of these two surgical approaches.

  11. 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.

  12. Islamic Architecture and Arch

    Directory of Open Access Journals (Sweden)

    Mohammed Mahbubur Rahman

    2015-01-01

    Full Text Available The arch, an essential architectural element since the early civilizations, permitted the construction of lighter walls and vaults, often covering a large span. Visually it was an important decorative feature that was trans-mitted from architectural decoration to other forms of art worldwide. In early Islamic period, Muslims were receiving from many civilizations, which they improved and re-introduced to bring about the Renaissance. Arches appeared in Mesopotamia, Indus, Egyptian, Babylonian, Greek and Assyrian civilizations; but the Romans applied the technique to a wide range of structures. The Muslims mastered the use and design of the arch, employed for structural and functional purposes, progressively meeting decorative and symbolic pur-poses. Islamic architecture is characterized by arches employed in all types of buildings; most common uses being in arcades. This paper discusses the process of assimilation and charts how they contributed to other civilizations.

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

    Science.gov (United States)

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

    2011-04-01

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

  14. Repair of left coronary artery aneurysm, recurrent ascending aortic aneurysm, and mitral valve prolapse 19 years after Bentall's procedure in a patient with Marfan syndrome.

    Science.gov (United States)

    Badmanaban, Balaji; Mallon, Peter; Campbell, Norman; Sarsam, Mazin A I

    2004-01-01

    A 45-year-old female with Marfan syndrome had a Bentall's procedure performed 19 years ago. She presented with a 4-year history of gradually worsening dyspnea and decreasing exercise tolerance. Investigations revealed severe mitral valve prolapse, a left main stem coronary artery (LMSCA) aneurysm, and a recurrent aneurysm of the ascending aorta. The mitral valve was replaced and the aortic aneurysmal sac and the LMSCA aneurysm were then repaired by a modified Bentall procedure. The patient made an uneventful recovery and was discharged home.

  15. Personalised external aortic root support (PEARS) in Marfan syndrome: Analysis of 1-9 year outcomes by intention-to-treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement

    NARCIS (Netherlands)

    T. Treasure (Tom); J.J.M. Takkenberg (Hanneke); T. Golesworthy (Tal); F. Rega (Filip); M. Petrou (Mario); U. Rosendahl (Ulrich); R. Mohiaddin (Raad); M. Rubens (Michael); W. Thornton (Warren); B. Lees (Belinda); J. Pepper (John)

    2014-01-01

    textabstractObjective: Among people with Marfan syndrome who have a typical aortic root aneurysm, dissection is a characteristic cause of premature death. To pre-empt Type A dissection, composite root replacement with a mechanical valve became the standard of care in the 1980s and 1990s. This is

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

    OpenAIRE

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

    2011-01-01

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

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

    International Nuclear Information System (INIS)

    Veldhoen, Simon; Behzadi, Cyrus; Derlin, Thorsten; Henes, Frank Oliver; Adam, Gerhard; Bannas, Peter; Rybczinsky, Meike; Kodolitsch, Yskert von; Sheikhzadeh, Sara; Bley, Thorsten Alexander

    2015-01-01

    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.)

  18. 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.)

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

    Science.gov (United States)

    Veldhoen, Simon; Behzadi, Cyrus; Derlin, Thorsten; Rybczinsky, Meike; von Kodolitsch, Yskert; Sheikhzadeh, Sara; Henes, Frank Oliver; Bley, Thorsten Alexander; Adam, Gerhard; Bannas, Peter

    2015-03-01

    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.

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

    Science.gov (United States)

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

    2015-12-01

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

  1. 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...

  2. 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.

  3. 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.

  4. Improving risk assessment for post-surgical low cardiac output syndrome in patients without severely reduced ejection fraction undergoing open aortic valve replacement. The role of global longitudinal strain and right ventricular free wall strain

    NARCIS (Netherlands)

    Balderas-Munoz, K.; Rodriguez-Zanella, H.; Fritche-Salazar, J. F.; Avila-Vanzzini, N.; Juarez Orozco, L. E.; Arias-Godinez, J. A.; Calvillo-Arguelles, O.; Rivera-Peralta, S.; Sauza-Sosa, J. C.; Ruiz-Esparza, M. E.; Bucio-Reta, E.; Rmero, A.; Espinola-Zavaleta, N.; Dominguez-Mendez, B.; Gaxiola-Macias, M.; Martinez-Rios, M. A.

    2017-01-01

    Low cardiac output syndrome (LCOS) after surgical aortic valve replacement (SAVR) is related to increased mortality and treatment related costs. We aimed to evaluate whether echocardiography-derived left ventricular global longitudinal strain (LV-GLS) relates to the occurrence of postoperative LCOS

  5. 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.

  6. 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

    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....... 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...

  7. Electrothermally Tunable Arch Resonator

    KAUST Repository

    Hajjaj, Amal Z.

    2017-03-18

    This paper demonstrates experimentally, theoretically, and numerically a wide-range tunability of electrothermally actuated microelectromechanical arch beams. The beams are made of silicon and are intentionally fabricated with some curvature as in-plane shallow arches. An electrothermal voltage is applied between the anchors of the beam generating a current that controls the axial stress caused by thermal expansion. When the electrothermal voltage increases, the compressive stress increases inside the arch beam. This leads to an increase in its curvature, thereby increasing its resonance frequencies. We show here that the first resonance frequency can increase monotonically up to twice its initial value. We show also that after some electrothermal voltage load, the third resonance frequency starts to become more sensitive to the axial thermal stress, while the first resonance frequency becomes less sensitive. These results can be used as guidelines to utilize arches as wide-range tunable resonators. Analytical results based on the nonlinear Euler Bernoulli beam theory are generated and compared with the experimental data and the results of a multi-physics finite-element model. A good agreement is found among all the results. [2016-0291

  8. Value and limitations of chimney grafts to treat arch lesions.

    Science.gov (United States)

    Mangialardi, N; Ronchey, S; Malaj, A; Fazzini, S; Alberti, V; Ardita, V; Orrico, M; Lachat, M

    2015-08-01

    The endovascular debranching with chimney stents provides a minimally invasive alternative to open surgery with readily available devices and has extended the option of endoluminal therapy into the realm of the aortic arch. But a critical observation at the use of this technique at the aortic arch is important and necessary because of the lack of long-term results and long term patency of the stents. Our study aims to review the results of chimney grafts to treat arch lesions. A systematic health database search was performed in December 2014 according to the Prisma Guidelines. Papers were sought through a meticulous search of the MEDLINE database (National Library of Medicine, Bethesda, MA) using the Pubmed search engine. Twenty-two articles were eligible for detailed analysis and data extraction. A total of 182 patients underwent chimney techniques during TEVAR (Thoracic Endovascular Aneurysm Repair). A total of 217 chimney grafts were implanted: 36 to the IA, 1 to the RCCA, 91 to the LCCA and 89 to the LSA. The type of stent-graft used for TEVAR was described in 132 patients. The type and name of chimney graft was described in 126 patients. In 53 patients information was limited to the type. Primary technical success, defined as a complete chimney procedure was achieved in 171 patients (98%). In 8 patients it was not clearly reported. The overall stroke rate was 5.3%. The overall endoleak rate, in those papers were it was clearly reported, was 18.4% (31 patients); 23(13,6%) patients developed a type IA endoleak, 1 patient (0.6%) developed type IB endoleak and 7 patients (4.1%) developed a type II endoleak The total endovascular aortic arch debranching technique represent a good option to treat high-risk patients, because it dramatically reduces the aggressiveness of the procedure in the arch. Many concerns are still present, mainly related to durability and material interaction during time. Long-term follow-up is exceptionally important in light of the

  9. 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.

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

    NARCIS (Netherlands)

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

    2008-01-01

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

  11. Surgical treatment of chronic multivascular mesenteric ischemia in a patient with antiphospholipid syndrome, abdominal aortic aneurism, and renal cancer: when planning overwhelms complexity.

    Science.gov (United States)

    Paliogiannis, Panagiotis; Ginesu, Giorgio Carlo; Feo, Claudio Francesco; Cossu, Maria Laura; Pinna, Antonio; Farina, Giulia; Vidili, Gianpaolo; Porcu, Alberto

    2016-12-20

    Chronic mesenteric ischemia is a clinical condition caused by obstructive or occlusive disease of the mesenteric vessels, with potentially lethal consequences. We describe a case of open multiple revascularization in a patient affected by antiphospholipid syndrome and diffuse atherosclerosis, with an abdominal aortic aneurism, a contracted kidney, a renal cancer affecting the contralateral kidney, and as a consequence, a chronic renal failure and hypertension. We revascularized the celiac trunk, the superior and inferior mesenteric arteries, and the right renal artery using saphenous grafts; the aneurism was corrected, and the renal tumor was treated by radiofrequency ablation. Despite the invasiveness and complexity, the surgical strategy adopted allowed to save the patient's life, to treat the chronic mesenteric ischemia and the renal cancer, and to improve the chronic renal insufficiency and hypertension. Graft, Mesenteric ischemia, Occlusion, Revascularization.

  12. Dental Arch Wire

    Science.gov (United States)

    1979-01-01

    Straightening teeth is an arduous process requiring months, often years, of applying corrective pressure by means of arch wires-better known as brace-which may have to be changed several times in the course of treatment. A new method has been developed by Dr. George Andreasen, orthodontist and dental scientist at the University of Iowa. The key is a new type of arch wire material, called Nitinol, with exceptional elasticity which helps reduce the required number of brace changes. An alloy of nickel and titanium, Nitinol was originally developed for aerospace applications by the Naval Ordnance Laboratory, now the Naval Surface Weapons Laboratory, White Oaks, Maryland. NASA subsequently conducted additional research on the properties of Nitinol and on procedures for processing the metal.

  13. 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/

  14. Aorto-aortic intrathoracic bypass in surgical treatment of aortic

    International Nuclear Information System (INIS)

    Gutierrez Perez, F.; Duran Reyes, A.; Bigalli, D.; Filgueira Berobide, J.

    1998-01-01

    The prevalence of coarctation of the aorta is 6.5 percent of all congenital heart defects, according to national and international data. There is a restenosis rate of patients after surgery. Factors that influence this evolution depends on the age at which patients underwent surgery for the first time the anatomy of the aortic arch and type of surgical technique. Several procedures can be used to correct the coarctation, which include surgery and balloon catheter dilation. We present here a case of a patient of 22 years old, with a recurrent coarctation of the aorta studied by echocardiography and magnetic resonance imaging. The patient underwent surgery a third time. We used an anterior approach (median sternotomy) and performed an aortic bypass graft, intrathoracic, under cardiopulmonary bypass. Evolved favorably and was discharged on the sixth day of post operative day (Author) [es

  15. Oclusão Completa do Arco Aórtico em Paciente Portadora de Arterite de Takayasu e Espondilite Anquilosante / Total Aortic arch Occlusion in a Patient with Takayasu's Arteritis and Spondylitis Ankylosans

    Directory of Open Access Journals (Sweden)

    Rodolfo Souza Cardoso

    2015-03-01

    stiffness of the spine, headache, dizziness, absence of pulses in the upper limbs and systemic hypertension. The CT angiography revealed occlusion of the aorta of the supra-aortic trunks (SAT and signals of spondylopathy, characterized by syndesmophytes between vertebral bodies and signs of ankylosis of the sacroiliac joint. The digital subtraction angiography confirmed a complete occlusion of the TSA in its origins and extensive collateral circulation. Treatment was started with immunosuppressants and corticosteroids, and treatment of hypertension, with a reduction in blood pressure and improvement of headache, although a maintenance of low back pain and joint stiffness was observed. Conclusion: The simultaneous occurrence of two seemingly distinct diseases such as TA and AS should be int