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Sample records for aortic hypoplasia presented

  1. Brainstem hypoplasia presenting with mirror movement

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    Burcu Ekmekçi

    2015-09-01

    Full Text Available 20 years old female patient, who had operated from congenital syndactyly on her left hand at five age, admitted to neurology policlinic with involuntary movement on her hands. We saw mirror movement (MM when she writing, catching with her left hand. This movement is had low amplitude in the right hand than left. Cervical MRG revealed no abnormality. Brain MRG revealed right middle, inferior cerebellary peduncle, olive and pyramid hypoplasia. Mirror movement shows homolog muscle activity which simulating contralateral movement, during a spesific task. This movement is seen usually upper extremity especially in the hand. Corticospinal tract dysfunction is often considered in the pathogenesis. MM may present as part of cervico medullary junction abnormality, cerebral palsy, cerebrovasculary disease, Parkinson disease. We wanted to discuss the patogenesis of MM in our patient with syndactyly and MRG abnormality.

  2. Adrenal Hypoplasia Congenita Presenting as Congenital Adrenal Hyperplasia

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    Jennifer L. Flint

    2013-01-01

    Full Text Available We report on a patient with genetically confirmed adrenal hypoplasia congenita (AHC whose presentation and laboratory abnormalities were consistent with the more common condition, congenital adrenal hyperplasia (CAH. The patient presented with failure to thrive and salt wasting. General appearance showed marked hyperpigmentation and normal male genitalia. He displayed mildly elevated 17-hydroxyprogesterone and markedly elevated 11-deoxycortisol levels at baseline and with ACTH stimulation testing. Results were consistent with 11β-hydroxylase deficiency. He required glucocorticoids and high doses of mineralocorticoids. The marked elevation in 11-deoxycortisol directed our clinical reasoning away from a hypoplastic condition and towards a hyperplasic adrenal condition. Sequencing of the DAX1 gene (named for dosage-sensitive sex reversal (DSS locus and the AHC locus on the X chromosome revealed a missense mutation. A review of the literature revealed that elevated 11-deoxycortisol levels have been noted in kindreds with DAX1 mutations, but only when measured very early in life. A mouse model has recently been described that displays elevated 11-deoxycorticosterone levels and evidence for hyperplasia of the zona glomerulosa of the adrenal gland. We conclude that DAX1 testing may be considered in patients with laboratory evidence of 11β-hydroxylase deficiency, especially in those with severe salt wasting.

  3. A Rare Presentation of Right Lung Hypoplasia Associated with Dextrocardia and Visceral Malposition

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    Ayşe Yıldırım

    2012-08-01

    Full Text Available Lung hypoplasia is often associated with pulmonary venous return abnormalities, referred to as the Scimitar syndrome, in pediatric patients. A two day-old male patient presented to our clinic with respiratory distress and mild cyanosis. Diagnostic studies revealed dextrocardia, right sided hypoplasia of upper and middle lung lobes and enlargement of the left lung due to the compensation, midline liver, right sided stomach and right sided spleen. No pulmonary venous return abnormalities were detected. This is the first report of lung hypoplasia associated with heterotaxy, visceral malposition and normal pulmonary venous return.

  4. Hypoplasia of the thumb. Clinical presentation and reconstruction

    International Nuclear Information System (INIS)

    In the genesis of the partial or total absence of the thumb they are genetic, environmental factors or a combination of both. It is take part of a syndrome or to be isolated and frequently associated with problems of radial longitudinal deficiency of the forearm. Objective. The purpose of this study is shown the experience, the focus of the processing and the results obtained since the point esthetic and functional view. The most it accepted classification is the proposal by Blauth that helps to determine the forecast and the processing. Materials and methods. it is a work type series of cases in 22 children with hypoplasia of the thumb, with a minimum of 12 months, (average 28 months). In 15 cases there were association of radial dysplasia or another anomaly among them 4 patients with VATER, and the 7 remaining they corresponded to hypoplasia of the thumb as only entity. We carried out tendon transfer, with opening of the first comisure in 2 patients with hypoplasia type II. In 3 patients, with hypoplasia type III A, one carries out corner opening, transfer of the superficial flexor of the 4 finger to correct instability of the articulation MF and opposition of the thumb, and transfer for extension of the thumb. In 17 cases one carries out politicization of the index. Results. The outcome was evaluated in: non pinch, lateral pinch and fingertip pinch; the grade of opposition like good, minimal and non opposition, and the aesthetic result according to the satisfaction of the parents in bad, regular and good. The five children reconstructed with transfers of tendons and comisure opening had good result. In 17 children with politicization one patient had a necrosis, of the 16 remaining a good or acceptable result was obtained. Discussion. It is not easy to follow a good system of measure of the functional results. We find that a practical way to evaluate was the clip, opposition and aesthetics. Previous to the surgeries it is required to evaluate alterations

  5. Aortic Dissection Cases Presenting with Neurological Deficit

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    Sibel Mumcu

    2014-06-01

    Full Text Available Aortic dissection can be fatal in case of underdiagnosis, and early treatment is essential for the survival. Although acute onset of chest or back pain is most common presenting symptoms, some patients might present with atypical findings such as acute stroke, mesenteric ischaemia, renal failure or myocardial infarction. Here, we report two cases presenting emergency room with atypical findings of aortic dissection which diagnosis is made during etiologic work up for ischemic stroke.

  6. [Abdominal aortic aneurysm: an uncommon presentation].

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    Taborda, Lúcia; Pereira, Laurinda; Amona, Eurides; Pinto, Erique Guedes; Rodrigues, Joaquim

    2011-01-01

    Most abdominal aortic aneurysms are asymptomatic, being accidentally found on physical examination or in routinely performed imaging studies. They only require surveillance (which is variable according to the aneurism size) and medical therapy in order to achieve risk factor reduction. However, in certain situations, according to the risk of aneurism rupture, elective surgery or endovascular procedure may be necessary. About 80% of the cases of aneurism rupture occur into the retroperitoneal space, with a high mortality rate. There are uncommon presentations of aneurism rupture as the aorto-caval fistula, which also require fast diagnosis and intervention. The authors present the case of a 71-year-old man, with the previous diagnosis of hypertension, acute myocardial infarction 2 months earlier (undergone primary Percutaneous Coronary Intervention) and tabagism, who was admitted at the emergency department with intense 24-hour-evolution epigastric pain. On physical examination, the Blood Pressure values measured at the lower limbs were about half the ones measured at the upper limbs and there was an abdominal pulsatile mass, with a high-intensity murmur. As the authors suspected aortic dissection, aneurysm, coarctation or thrombosis, it was done a Computed Tomography scanning with intravenous contrast, which revealed a ruptured abdominal aorta aneurysm with a mural thrombus. The doppler ultrasound confirmed the presence of a high debit aorto-caval fistula. The patient was immediately transferred to the Vascular Surgery. However he died 2 hours later, during surgery. PMID:22525642

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

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    Kafka, Henryk; Uebing, Anselm; Mohiaddin, Raad

    2006-11-01

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

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

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

    2014-01-01

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

  9. Pancreatic hypoplasia presenting with neonatal diabetes mellitus in association with congenital heart defect and developmental delay.

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    Balasubramanian, M; Shield, J P H; Acerini, C L; Walker, J; Ellard, S; Marchand, M; Polak, M; Vaxillaire, M; Crolla, J A; Bunyan, D J; Mackay, D J G; Temple, I K

    2010-02-01

    Congenital pancreatic hypoplasia is a rare cause of neonatal diabetes. We report on a series of three patients with pancreatic agenesis and congenital heart defects. All had abdominal scan evidence of pancreatic agenesis. In addition, Patient 1 had a ventricular septal defect, patent ductus arteriosus and pulmonary artery stenosis; Patient 2 had a truncus arteriosus and Patient 3 had tetralogy of Fallot. Two of the three patients have developmental delay. All three patients were isolated cases within the family. Investigations included sequencing of GCK, ABCC8, IPF1, NEUROD1, PTF1A, HNF1B, INS, ISL1, NGN3, HHEX, G6PC2, TCF7L2, SOX4, FOXP3 (Patients 1 and 2), GATA4 and KCNJ11 genes (all three patients), but no mutations were found. Genetic investigation to exclude paternal UPD 6, methylation aberrations and duplications of 6q24 was also negative in all three. 22q11 deletion was excluded in all three patients. Array CGH in Patient (1) showed a approximately 250 kb, paternally inherited duplication of chromosome 12q [arr cgh 12q24.33 (B35:CHR12:131808577-132057649++) pat], not found in the other two patients. Permanent neonatal diabetes mellitus due to pancreatic hypoplasia with congenital heart defects has been reported before and may represent a distinct condition. We discuss this rare association and review previously reported literature. PMID:20082465

  10. Acute aortic occlusion presenting as flaccid paraplegia.

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    Kilany, Ayman; Al-Hashel, Jasem Y; Rady, Azza

    2015-01-01

    A 67-year-old male known to be hypertensive and diabetic had a sudden onset of severe low back pain and flaccid paraplegia with no sensory level or bladder affection and the distal pulsations were felt. Acute compressive myelopathy was excluded by MRI of the dorsal and lumbar spines. The nerve conduction study and CSF analysis was suggestive of acute demyelinating polyneuropathy. The patient developed ischemic changes of the lower limb and CT angiography revealed severe stenosis of the abdominal aorta and both common iliac arteries. We emphasize the importance of including acute aortic occlusion in the differential diagnosis of acute flaccid paraplegia especially in the presence of severe back pain even if the distal pulsations were felt. PMID:25866688

  11. Cerebellar Hypoplasia

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    ... disorders that begin in early childhood, such as ataxia telangiectasia. In an infant or young child, symptoms of a disorder that features cerebellar hypoplasia might include floppy muscle tone, developmental or ...

  12. Giant Aortic Root Aneurysm Presenting as Acute Type A Aortic Dissection

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    Raz, Guy M.; Stamou, Sotiris C.

    2014-01-01

    A 49-year-old woman with four months of increasing episodic palpitations, chest pain, and shortness of breath presented to an outside clinic where a new 4/6 systolic ejection murmur was identified. A transthoracic echocardiogram revealed a large aortic root aneurysm. The patient underwent emergent repair of the dissected root aneurysm with a modified Bentall procedure utilizing a #19 St Jude Valsalva mechanical valve conduit. Postoperatively, she required a permanent pacemaker placement. Her ...

  13. Acute Paraplegia as a Presentation of Aortic Saddle Embolism

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    Lisandro Irizarry

    2016-01-01

    Full Text Available Background. Acute onset paraplegia has a myriad of causes most often of a nonvascular origin. Vascular etiologies are infrequent causes and most often associated with postsurgical complications. Objective. To describe the occurrence and possible mechanism for aortic saddle embolism as a rare cause of acute paraplegia. Case Report. Described is a case of a 46-year-old female who presented with the sudden onset of nontraumatic low back pain with rapidly progressive paraplegia which was subsequently determined to be of vascular origin.

  14. Crystalline Ultrastructures, Inflammatory Elements, and Neoangiogenesis Are Present in Inconspicuous Aortic Valve Tissue

    OpenAIRE

    2010-01-01

    Morbidity from calcific aortic valve disease (CAVD) is increasing. Recent studies suggest early reversible changes involving inflammation and neoangiogenesis. We hypothesized that microcalcifications, chemokines, and growth factors are present in unaffected regions of calcific aortic valves. We studied aortic valves from 4 patients with CAVD and from 1 control, using immunohistochemistry, scanning electron microscopy, and infrared spectrography. We revealed clusters of capillary neovessels in...

  15. Crystalline Ultrastructures, Inflammatory Elements, and Neoangiogenesis Are Present in Inconspicuous Aortic Valve Tissue

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    P. Dorfmüller; Bazin, D.(National Superconducting Cyclotron Laboratory, Department of Physics and Astronomy, Michigan State University, East Lansing, MI, 48824, USA); Aubert, S; Weil, R.; Brisset, F.; Daudon, M.; Capron, F; Brochériou, I

    2010-01-01

    Morbidity from calcific aortic valve disease (CAVD) is increasing. Recent studies suggest early reversible changes involving inflammation and neoangiogenesis. We hypothesized that microcalcifications, chemokines, and growth factors are present in unaffected regions of calcific aortic valves. We studied aortic valves from 4 patients with CAVD and from 1 control, using immunohistochemistry, scanning electron microscopy, and infrared spectrography. We revealed clusters of capillary neovessel...

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

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    毛乐; 祝忠群

    2016-01-01

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

  17. Mycotic aneurysm of the aortic arch presenting with left vocal cord palsy.

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    Tokmaji, George; Gosev, Igor; Kumamaru, Kanako Kunishima; Bolman, Ralph Morton

    2013-07-01

    We report a case of a 71-year-old man with a mycotic aneurysm of the aortic arch who presented with progressive hoarseness. Three weeks prior to this event the patient was admitted to an outside hospital in septic condition and was diagnosed with a mycotic abdominal aortic aneurysm. Resection of the infected abdominal aortic aneurysm with right axillofemoral and femoral-femoral bypass grafts was performed and the patient was discharged home on intravenous antibiotics. At our institution, the aortic arch aneurysm was treated with extensive debridement and replaced with a Dacron prosthesis under circulatory arrest with antegrade cerebral perfusion through the axillofemoral bypass. PMID:23816081

  18. [Ruptured abdominal aortic aneurysm. A rare form of presentation].

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    Rettedal, E A; Vennesland, O

    1993-05-10

    In most cases a ruptured abdominal aortic aneurism is dramatic, with rapid deterioration of the clinical condition of the patient. With abdominal and back pain, pulsatile tumour, and development of bleeding shock the diagnosis is obvious. In some cases the symptoms are not clear and the condition can be misinterpreted. The authors describe a case to illustrate this. A 74 year-old male was admitted to hospital with vague abdominal pain and left inguinal hernia. It later turned out that a ruptured abdominal aortic aneurism was the reason for his symptoms and signs. 14 similar cases are reported in the literature. PMID:8332976

  19. Temporoparietal Headache as the Initial Presenting Symptom of a Massive Aortic Dissection

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    Manan Parikh

    2015-01-01

    Full Text Available Aortic dissection is a life-threatening medical emergency often presenting with severe chest pain and acute hemodynamic compromise. The presentation of aortic dissection can sometimes be different thus leading to a challenge in prompt diagnosis and treatment as demonstrated by the following presentation and discussion. We present a case of a 71-year-old male who presented to the emergency department with complaints of left sided temporoparietal headache and was eventually diagnosed with a thoracic aortic dissection involving the ascending aorta and descending aorta, with an intramural hematoma in the descending aorta. This case illustrates the importance of keeping in mind aortic dissection as a differential diagnosis in patients with acute onset headaches in which any intracranial source of headache is not found.

  20. A rare presentation of late right coronary artery spasm following aortic valve replacement

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    Alizadeh-Ghavidel, Alireza; Basiri, Hosseinali; Totonchi, Ziae; Mirmesdagh, Yalda; Jalili-Shahandashti, Farshad; Gholizadeh, Behnam

    2015-01-01

    BACKGROUND Coronary artery spasm (CAS) is defined as a reversible, sudden epicardial coronary artery stenosis that causes vessel occlusion or near occlusion. CASE REPORT In this article, we present a clinical case of CAS in a 48-year-old woman undergoing elective aortic valve replacement surgery for aortic stenosis. On the 3rd post-operative day, the patient suffered from chest pain and dyspnea. Emergent coronary angiography demonstrated a significant spasm of the ostium portion of the right ...

  1. An endoluminal aortic prosthesis infection presenting as pneumoaorta and aortoduodenal fistula

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    Yung-Ta Kao; Chun-Ming Shih; Feng-Yen Lin; Nai-Wen Tsao; Nen-Chung Chang; Chun-Yao Huang

    2012-01-01

    Herein,we present a case of pneumoaorta and aortoduodenal fistula (ADF) caused by an endoluminal aortic prosthesis infection.An 82-year-old man underwent endovascular aneurysm repair with a stent graft to exclude a 5.1-cm abdominal aortic aneurysm.Three months after the index procedure,the patient was taken to the emergency department at a medical university hospital.He presented with a 2-d history of bloody diarrhea.An endoluminal aortic stent graft infection was diagnosed,and an ADF was identified.The patient died of septic shock despite emergency surgery and intensive care.When encountered,stent graft infections require appropriate antibiotics and graft explantation.The diagnosis of an ADF is important,and surgery remains the most effective management if septic shock presents despite conservative treatment.

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

    International Nuclear Information System (INIS)

    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

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

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    Sharon H. Gnagi

    2015-01-01

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

  4. New insights regarding the incidence, presentation and treatment options of aorto-oesophageal fistulation after thoracic endovascular aortic repair

    DEFF Research Database (Denmark)

    Czerny, Martin; Eggebrecht, Holger; Sodeck, Gottfried;

    2014-01-01

    To review the incidence, clinical presentation, definite management and 1-year outcome in patients with aorto-oesophageal fistulation (AOF) following thoracic endovascular aortic repair (TEVAR).......To review the incidence, clinical presentation, definite management and 1-year outcome in patients with aorto-oesophageal fistulation (AOF) following thoracic endovascular aortic repair (TEVAR)....

  5. MR findings in pontocerebellar hypoplasia

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    Uhl, M.; Pawlik, H.; Laubenberger, J.; Langer, M. [Department of Radiology, Division of Paediatric Radiology, University Hospital of Freiburg, Freiburg (Germany); Darge, K. [Department of Paediatric Radiology, Children`s Hospital of the University of Heidelberg, Heidelberg (Germany); Baborie, A. [Department of Neuropathology, Neurozentrum, University of Freiburg, Freiburg (Germany); Korinthenberg, R. [Department of Neuropaediatrics, Children`s Hospital, University of Freiburg, Freiburg (Germany)

    1998-07-01

    We present four cases with combined hypoplasia of the cerebellum and the ventral pons - pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to `float` in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect. (orig.) With 3 figs., 2 tabs., 13 refs.

  6. Optic nerve hypoplasia

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    Savleen Kaur

    2013-01-01

    Full Text Available Optic nerve hypoplasia (ONH is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65% than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED.

  7. Optic nerve hypoplasia.

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    Kaur, Savleen; Jain, Sparshi; Sodhi, Harsimrat B S; Rastogi, Anju; Kamlesh

    2013-05-01

    Optic nerve hypoplasia (ONH) is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65%) than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED). PMID:24082663

  8. Inflammatory abdominal aortic aneurysm presenting as bilateral hydroureteronephrosis: A case report and review of literature

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    Andrea Benedetto Galosi; Carlo Grilli Cicilioni; Giulia Sbrollini; Andrea Angelini; Guevar Maselli; Luciano Carbonari

    2014-01-01

    We report a case of Inflammatory Abdominal Aortic Aneurysm (IAAA) producing bilateral hydro-ureteronephrosis. A 74-year-old patient presented to urologist office for bilateral hydronephrosis detected by kidney and bladder ultrasound (US). Patient reported lower urinary tract symptoms and inconstant and slight low back pain irradiated to inguinal region dating 3 weeks. Renal function, urine analysis and abdominal examination were normal. However the repeated ultrasound in the urologist office ...

  9. Inflammatory abdominal aortic aneurysm presenting as bilateral hydroureteronephrosis: a case report and review of literature.

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    Galosi, Andrea Benedetto; Grilli Cicilioni, Carlo; Sbrollini, Giulia; Angelini, Andrea; Maselli, Guevar; Carbonari, Luciano

    2014-12-01

    We report a case of Inflammatory Abdominal Aortic Aneurysm (IAAA) producing bilateral hydro-ureteronephrosis. A 74-year-old patient presented to urologist office for bilateral hydronephrosis detected by kidney and bladder ultrasound (US). Patient reported lower urinary tract symptoms and inconstant and slight low back pain irradiated to inguinal region dating 3 weeks. Renal function, urine analysis and abdominal examination were normal. However the repeated ultrasound in the urologist office revealed abdominal aortic aneurism extended to iliac vessels. The patient was sent directly to vascular surgery unit where contrast computerized tomography (CT) and successful surgical repair were done. Final diagnosis was IAAA. The post-operative course was uneventful. Renal function was regular and the hydronephrosis reduced spontaneously under monitoring by CT and US. We review diagnosis and management of hydronephrosis that is sometimes linked to IAAA rather than standard AAA. Abdominal ultrasound is mandatory in any bilateral hydronephrosis and it could save lives. PMID:25641477

  10. Inflammatory abdominal aortic aneurysm presenting as bilateral hydroureteronephrosis: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Andrea Benedetto Galosi

    2014-12-01

    Full Text Available We report a case of Inflammatory Abdominal Aortic Aneurysm (IAAA producing bilateral hydro-ureteronephrosis. A 74-year-old patient presented to urologist office for bilateral hydronephrosis detected by kidney and bladder ultrasound (US. Patient reported lower urinary tract symptoms and inconstant and slight low back pain irradiated to inguinal region dating 3 weeks. Renal function, urine analysis and abdominal examination were normal. However the repeated ultrasound in the urologist office revealed abdominal aortic aneurism extended to iliac vessels. The patient was sent directly to vascular surgery unit where contrast computerized tomography (CT and successful surgical repair were done. Final diagnosis was IAAA. The post-operative course was uneventful. Renal function was regular and the hydronephrosis reduced spontaneously under monitoring by CT and US. We review diagnosis and management of hydronephrosis that is sometimes linked to IAAA rather than standard AAA. Abdominal ultrasound is mandatory in any bilateral hydronephrosis and it could save lives.

  11. Acquired post-traumatic aortic coarctation presenting as new-onset congestive heart failure: treatment with endovascular repair.

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    Thompson, Norris B; Hamidian Jahromi, Alireza; Ballard, David H; Rao, Vyas R; Samra, Navdeep S

    2015-01-01

    Acquired coarctation of the thoracic aorta is a rare phenomenon in adults. The etiology is often idiopathic, but severe stenosis can develop from prior surgery, blunt thoracic aortic injuries, or severe atherosclerotic/atheroembolic disease. Common symptomatic presentations include refractory upper extremity hypertension and new-onset congestive heart failure. We present the case of a 52-year-old man who developed acquired thoracic aortic coarctation 30 years after a blunt trauma and deceleration injuries to the aorta requiring open surgical aortic repair. He presented with poorly controlled hypertension and new-onset heart failure and was treated surgically with endovascular repair.

  12. Crystalline Ultrastructures, Inflammatory Elements, and Neoangiogenesis Are Present in Inconspicuous Aortic Valve Tissue

    Directory of Open Access Journals (Sweden)

    P. Dorfmüller

    2010-01-01

    Full Text Available Morbidity from calcific aortic valve disease (CAVD is increasing. Recent studies suggest early reversible changes involving inflammation and neoangiogenesis. We hypothesized that microcalcifications, chemokines, and growth factors are present in unaffected regions of calcific aortic valves. We studied aortic valves from 4 patients with CAVD and from 1 control, using immunohistochemistry, scanning electron microscopy, and infrared spectrography. We revealed clusters of capillary neovessels in calcified (ECC, to a lesser extent in noncalcified (ECN areas. Endothelial cells proved constant expression of SDF-1 in ECC, ECN, and endothelial cells from valvular surface (ECS. Its receptor CXCR4 was expressed in ECC. IL-6 expression correlated with CXCR4 staining and presence of lymphocytes. VEGF was expressed by ECS, its receptor by ECC and ECN. Crystalline ultrastructures were found on the surface of histologically noncalcified areas (HNCAs, spectrography revealed calcium hydroxylapatite. Our results demonstrate that crystalline ultrastructures are present in HNCAs, undergoing neoangiogenesis in an inflammatory context. These alterations could be an early witness of disease and an opening to therapy.

  13. Chronic contained rupture of an abdominal aortic aneurysm presenting as a Grynfeltt lumbar hernia. A case report.

    Science.gov (United States)

    Dobbeleir, J; Fourneau, I; Maleux, G; Daenens, K; Vandekerkhof, J; Nevelsteen, A

    2007-06-01

    We report a unique case of chronic contained thoraco-abdominal aneurysm rupture presenting as a Grynfeltt lumbar hernia. A 79-year-old man presented with backpain and a bluish swelling in the left lumbar region in the presence of a non tender aortic aneurysm. CT scan confirmed contained rupture of a type IV thoraco-abdominal aortic aneurysm. The peri-aortic haematoma protruded through the lumbar wall causing a Grynfeltt lumbar hernia. The aneurysm was replaced through a thoraco-phreno-lumbotomy. The patient survived and is doing well six months postoperatively.

  14. Retalho de pericárdio pediculado vascularizado autógeno para aortoplastia e correção da coarctação simples de aorta torácica, ou associada à hipoplasia, atresia ou interrupção do arco aórtico Pediculated autologous vascularized pericardial flap aortoplasty for correction of simple aortic coarctation or associated with hypoplasia, atresia or interruption of aortic arch

    Directory of Open Access Journals (Sweden)

    Paulo Rodrigues da Silva

    2006-12-01

    . CONCLUSÃO: A técnica do emprego do retalho pericárdico pediculado, vascularizado e autógeno é a mais adequada e a mais completa em comparação com todas as outras técnicas cirúrgicas existentes para correção dos diferentes tipos de coarctação da aorta torácica, nas suas formas simples ou associadas a outras lesões aórticas. É indicada em todas as faixas etárias, inclusive em recém-natos.OBJECTIVE: Eighteen years ago, two young male patients of 8 months and 13 years with aortic coarctation associated to aortic hypoplasia between the left subclavian artery and the coarctated area, were submitted to surgical correction using a new world-pioneering surgical technique developed in our service. METHOD: This technique consists of sectioning the patent ductus arteriosus, followed by resection of all the coarctated tissue in the aortic wall and aortoplasty correction by means of the lengthwise implantation of a pediculated autogenous pericardial flap. This flap is inserted into the thoracic aorta, from the root of the left subclavian artery to 2.0 cm below the coarctated area. RESULTS: For both patients, the blood pressure and arterial pulses of both arms and legs have been normal since the surgery until the present moment. Clinical examination and Doppler evaluation evidenced no pressure gradient between arms and legs, normal blood flow and no pressure gradient through the coarctated area. Both patients were submitted to other evaluations 18 years after surgery, including cardiac and thoracic aortic catheterization followed by aortography. These evaluations demonstrated normal aortic configuration, with normal diameter, including the areas above and below the coarctated site. There was no evidence of any kind of degenerative lesions of the vascularized pericardial flap or re-coarctation of the lesion and no signs of aneurysms forming or the presence of atherosclerosis of the flap. Moreover, and very importantly, it was evident that the pediculated completely

  15. Fetal MRI and antenatal diagnosis of unilateral cerebellar hypoplasia

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    Houda El Mhabrech

    2015-03-01

    Full Text Available Focal cerebellar hypoplasia is restricted to one cerebellar hemisphere or to the vermis. Prenatal diagnosis of unilateral cerebellar hypoplasia is possible by the use of ultrasound and MRI. Familiarity with the prenatal MRI findings is essential to recognize cerebellar pathologies accurately and prospectively. We present US and MRI findings in a fetus with cerebellar malformation at 20 weeks gestation. The goal of our case report is to present the fetal MRI findings of unilateral cerebellar hypoplasia, to discuss the value of fetal MRI in the early diagnoses of this malformation and to summarize the current main stream literature concerning the etiology.

  16. Cerebral white matter hypoplasia

    International Nuclear Information System (INIS)

    This paper demonstrates the MR imaging findings in children with cerebral white matter hypoplasia (CWMH). The MR studies of four children, aged 3-7 y (mean age, 2.3 y) with a diagnosis of CWMH were reviewed. In all cases multiplanar T1-weighted and T2-weighted spin-echo images were obtained. All children had similar histories of severe developmental delay and nonprogressive neurologic deficits despite normal gestational and birth histories. In two cases there was a history of maternal cocaine abuse. Autopsy correlation was available in one child. The MR images of all four children demonstrated diffuse lack of white matter and enlarged ventricles but normal-appearing gray matter. The corpus callosum, although completely formed, was severely thinned. There was no evidence of gliosis or porencephaly, and the distribution of myelin deposition was normal for age in all cases. Autopsy finding in one child correlated exactly with the MR finding

  17. Aplasia and hypoplasia of the maxillary sinus: A case series

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    Nasim Jafari-Pozve

    2014-01-01

    Full Text Available Maxillary sinus aplasia and hypoplasia are rare conditions that can cause symptoms such as headaches and voice alteration. The majority of patients are asymptomatic, but these conditions must be noticed for importance of differential diagnosis such as infection and neoplasms. Conventional radiographs could not differentiate between inflammatory mucosal thickening, neoplasm, and hypoplasia of the sinus. Computed tomography (CT and also cone beam computed tomography (CBCT are the proper modalities to detect these conditions. In the present study, CBCT findings of three cases with maxillary sinus hypoplasia and aplasia are reported.

  18. Slowly Progressive and Painless Thoracic Aortic Dissection Presenting with a Persistent Fever in an Elderly Patient: The Usefulness of Combined Measurement of Biochemical Parameters

    Directory of Open Access Journals (Sweden)

    Shunsuke Yamada

    2013-01-01

    Full Text Available Aortic dissection is a fatal medical condition that requires urgent diagnosis and appropriate intervention. Because acute aortic dissection often manifests as sudden onset excruciating chest pain, physicians can easily reach a proper diagnosis. However, some patients with aortic dissection present with varied clinical manifestations without exhibiting typical chest pain, leading to a delayed diagnosis and possible fatality. We herein present the case of an elderly subject with a fever of unknown origin who was ultimately diagnosed with aortic dissection. In the present case, a negative procalcitonin test, increased D-dimer and serum creatinine phosphokinase-BB levels, and reelevation of the CPR level led us to the correct diagnosis.

  19. Fetal MRI and antenatal diagnosis of unilateral cerebellar hypoplasia

    OpenAIRE

    Houda El Mhabrech; Ahmed Zrig; Chiraz Hafsa

    2015-01-01

    Focal cerebellar hypoplasia is restricted to one cerebellar hemisphere or to the vermis. Prenatal diagnosis of unilateral cerebellar hypoplasia is possible by the use of ultrasound and MRI. Familiarity with the prenatal MRI findings is essential to recognize cerebellar pathologies accurately and prospectively. We present US and MRI findings in a fetus with cerebellar malformation at 20 weeks gestation. The goal of our case report is to present the fetal MRI findings of unilateral cerebellar h...

  20. Aberrant left pulmonary artery associated with right pulmonary hypoplasia

    International Nuclear Information System (INIS)

    Aberrant left pulmonary artery (ALPA), or pulmonary artery sling, is an uncommon vascular malformation that is frequently associated with obstructive disorders of the tracheobronquial tree. In newborns, it produces severe respiratory problems. In contrast, in adults, it is usually discovered by change. ALPA has been associated with right pulmonary hypoplasia (RPH) in a small number of cases. We present a new case of ALP associated with right pulmonary hypoplasia in an adult woman, diagnosed by CT and MR. 12 refs

  1. Chronic complete thrombosis of abdominal aortic aneurysm: an unusual presentation of an unusual complication.

    Science.gov (United States)

    Pejkic, Sinisa; Opacic, Dragan; Mutavdzic, Perica; Radmili, Oliver; Krstic, Nevena; Davidovic, Lazar

    2015-02-01

    Although mural thrombosis frequently accompanies aneurysmal disease, complete thrombosis is distinctly unusual complication of abdominal aortic aneurysm (AAA). A case study of a patient with chronic, asymptomatic complete thrombosis of a large juxtarenal AAA is presented along with a literature review and discussion of the potential secondary complications, mandating aggressive management of this condition. A 67-year-old man with multiple atherogenic risk factors and unattended complaints consistent with a recent episode of a transient right hemispheric ischemic attack was referred to our clinic with a diagnosis of a thrombosed AAA established by computed tomography. Duplex ultrasonography and aortography confirmed the referral diagnosis and also revealed near occlusion of the left internal carotid artery. The patient underwent a two-stage surgery, with preliminary left-sided carotid endarterectomy followed three days later by an aneurysmectomy and aortobifemoral reconstruction. He had an uncomplicated recovery and was discharged home on postoperative day 7, remaining asymptomatic at the 42-month follow-up. Complete thrombosis is an uncommon presentation of AAA and may be clinically silent. It is frequently associated with other manifestations of generalized atherosclerosis. Radical open repair yields durable result and is the preferred treatment modality.

  2. Lissencephaly and cerebellar hypoplasia in a goat

    Directory of Open Access Journals (Sweden)

    José Rômulo Soares dos Santos

    2013-10-01

    Full Text Available A case of lissencephaly and cerebellar hypoplasia was observed in a 30-day-old goat. The goat presented with sternal recumbence, absence of a menace response, intention tremors, ataxia, and nystagmus. The goat was euthanized and necropsied after having been hospitalised for eleven days. At necropsy, the surface of the brain was found to be smooth, the cerebral sulci and gyri were absent, and the cerebellum was reduced in size. Histologically, the grey matter and white matter were thicker and thinner than normal in cortices, respectively. The neurons were randomly arranged in the grey matter. In the cerebellum, the layers were disorganised, and cells were heterotopics. The histologic and gross lesions observed in this animal are characteristic of lissencephaly associated with cerebellar hypoplasia. The presence of a single goat affected suggests that the malformation was not of infectious origin and because lissencephaly is a malformation not previously described in goats, it is unlikely this case was inherited.

  3. Infected aortic aneurysm presenting as prevertebral abscess in magnetic resonance imaging: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Jung Re; Ko, Seong Joo; Heo, Sang Taek; Kim, Jin Seok; Kim, Seung Hyoung [Jeju National University School of Medicine, Jeju (Korea, Republic of)

    2013-09-15

    The differential diagnosis of mass-like lesions around the aorta includes saccular pseudoaneurysms and abscesses. A 69-year-old female was admitted with multiple abscesses and fluid collections in several muscles and joints. Methicillin susceptible Staphylococcus aureus was isolated from her blood and pus. Even though she was treated with repeated operations and appropriate antibiotics, her conditions worsened with persistent fever and newly developed abscesses at other sites. Serial enhanced computed tomography (CT) revealed a newly developed saccular abdominal aortic aneurysm in the retroaortic space at the celiac axis level. However, the infected aortic aneurysm mimicked a prevertebral abscess on enhanced spinal magnetic resonance imaging (MRI) due to its heterogeneous signal intensity caused by intraaneurysmal turbulent blood flow. We report to alert the pitfall in the diagnosis of saccular aortic aneurysm using spinal MRI and the importance of serial enhanced CT study in highly suspicious patients.

  4. Congenital Unilateral Hypoplasia of Depressor Anguli Oris

    Directory of Open Access Journals (Sweden)

    Seckin O. Ulualp

    2012-01-01

    Full Text Available Objectives. Asymmetric facial appearance may originate from abnormalities of facial musculature or facial innervation. We describe clinical features of congenital hypoplasia of depressor anguli oris muscle in a child. Material and Methods. Chart of a 10-month-old female referred to a tertiary care pediatric hospital for assessment of facial paralysis was reviewed. Data included relevant history and physical examination, diagnostic work up, and management. Results. The child presented with asymmetric movement of lower lip since birth. Asymmetry of lower lip was more pronounced when she smiled and cried. Rest of the face movement was symmetric. On examination, the face appeared symmetric at rest. The child had inward deviation of right lower lip when she smiled. Facial nerve function, as determined by frowning/forehead, wrinkling, eye closure, nasolabial fold depth, and tearing, was symmetric. Magnetic resonance imaging of the temporal bones and internal auditory canals were within normal limits. Echocardiogram did not show cardiac abnormality. Auditory brainstem response showed no abnormality. Conclusions. Congenital hypoplasia of depressor anguli oris is a rare anomaly that causes asymmetric crying face. Pediatricians and otolaryngologists need to be cognizant of cardiac, head and neck, and central nervous system anomalies associated with congenital unilateral hypoplasia of depressor anguli oris.

  5. Unusual Congenital Aortic Anomaly with Rare Common Celiamesenteric Trunk Variation: MR Angiography and Digital Substraction Angiography Findings

    International Nuclear Information System (INIS)

    Magnetic resonance angiography and digital substraction angiography (DSA) findings in a case with a rare congenital thoracoabdominal aortic hypoplasia and common celiamesenteric trunk variation with occlusion of infrarenal abdominal aorta are described here. To our knowledge, this aortic anomaly has not been previously described in the English literature. DSA is the optimum imaging modality for determination of aortic hypoplasia, associated vascular malformations, collateral vessels, and direction of flow within vessels

  6. Endovascular therapy for overcoming challenges presented with blunt abdominal aortic injury.

    Science.gov (United States)

    Gilani, Ramyar; Saucedo-Crespo, Hector; Scott, Bradford G; Tsai, Peter I; Wall, Metthew J; Mattox, Kenneth L

    2012-05-01

    Blunt abdominal aortic injury (BAAI) is a rare and lethal injury requiring surgical management. Injury patterns can be complex and surgical strategy should accommodate specific case circumstances. Endovascular solutions appear appropriate and preferred in certain cases of BAAI, which, however, may not be applicable due to device limitations in regard to patient anatomy and limited operating room capability. However, endovascular therapy can be pursued with limited fluoroscopy capability and consumable availability providing a solution that is expeditious and effective for select cases of BAAI.

  7. Review of numerical methods for simulation of the aortic root: Present and future directions

    Science.gov (United States)

    Mohammadi, Hossein; Cartier, Raymond; Mongrain, Rosaire

    2016-05-01

    Heart valvular disease is still one of the main causes of mortality and morbidity in develop countries. Numerical modeling has gained considerable attention in studying hemodynamic conditions associated with valve abnormalities. Simulating the large displacement of the valve in the course of the cardiac cycle needs a well-suited numerical method to capture the natural biomechanical phenomena which happens in the valve. The paper aims to review the principal progress of the numerical approaches for studying the hemodynamic of the aortic valve. In addition, the future directions of the current approaches as well as their potential clinical applications are discussed.

  8. Endovascular stent graft repair for thoracic aortic aneurysms: the history and the present in Japan.

    Science.gov (United States)

    Kawaguchi, Satoshi; Shimizu, Hideyuki; Yoshitake, Akihiro; Shimazaki, Taro; Iwahashi, Toru; Ogino, Hitoshi; Ishimaru, Shin; Shigematsu, Hiroshi; Yozu, Ryohei

    2013-01-01

    Stent-grafts for endovascular repair of thoracic aortic aneurysms have been commercially available for more than ten years in the West, whereas, in Japan, a manufactured stent-graft was not approved for the use until March 2008. Nevertheless, endovascular thoracic intervention began to be performed in Japan in the early 1990s, with homemade devices used in most cases. Many researchers have continued to develop their homemade devices. We have participated in joint design and assessment efforts with a stent-graft manufacturer, focusing primarily on fenestrated stent-grafts used in repairs at the distal arch, a site especially prone to aneurysm. In March 2008, TAG (W.L. Gore & Associates, Inc., Flagstaff, Arizona, USA) was approved as a stent graft for the thoracic area first in Japan, which was major turning point in treatment for thoracic aortic aneurysms. Subsequently, TALENT (Medtronic, Inc., Minneapolis, Minnesota, USA) was approved in May 2009, and TX2 (COOK MEDICAL Inc., Bloomington, Indiana, USA) in March 2011. Valiant as an improved version of TALENT was approved in November 2011, and TX2 Proform as an improved version of TX2 began to be supplied in October 2012. These stent grafts are excellent devices that showed good results in Western countries, and marked effectiveness can be expected by making the most of the characteristics of each device. A clinical trial in Japan on Najuta (tentative name) (Kawasumi Labo., Inc., Tokyo, Japan) as a line-up of fenestrated stent grafts that can be applied to distal arch aneurysms showing a high incidence, and allow maintenance of blood flow to the arch vessel was initiated. This trial was completed, and Najuta has just been approved in January of 2013 in Japan, and further development is expected. In the U.S., great efforts have recently been made to develop and manufacture excellent stent grafts for thoracic aneurysms, and rapid progress has been achieved. In particular, in the area of the aortic arch, in which we

  9. Congenital Medial Meniscus Hypoplasia.

    Science.gov (United States)

    Benedick, Alex; Shiraj, Sahar; Williams, James; Sundaram, Murali; Subhas, Naveen

    2016-05-01

    An 18-year-old man with no prior surgical history presented with left knee pain after sustaining a basketball injury and was diagnosed with transient patellar dislocation. Magnetic resonance imaging was ordered to further assess the extent of injury. PMID:27064776

  10. Aortic Annular Enlargement during Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Selman Dumani

    2016-09-01

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

  11. Aortic insufficiency

    Science.gov (United States)

    ... Heart valve - aortic regurgitation; Valvular disease - aortic regurgitation; AI - aortic insufficiency ... BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: ...

  12. Is vertebral artery hypoplasia a predisposing factor for posterior circulation cerebral ischemic events? A comprehensive review.

    Science.gov (United States)

    Katsanos, Aristeidis H; Kosmidou, Maria; Kyritsis, Athanassios P; Giannopoulos, Sotirios

    2013-01-01

    Vertebral artery hypoplasia is not currently considered an independent risk factor for stroke. Emerging evidence suggest that vertebral artery hypoplasia may contribute to posterior circulation ischemic events, especially when other risk factors coexist. In the present literature review, we present published data to discuss the relationship between a hypoplastic vertebral artery and posterior circulation cerebral ischemia. Despite difficulties and controversies in the accurate definition and prevalence estimation of vertebral artery hypoplasia, ultrasound studies reveal that the reduced blood flow observed ipsilateral to the hypoplastic vertebral artery may result in local cerebral hypoperfusion and subsequent focal neurological symptomatology. That risk of cerebral ischemia is related to the severity of the hypoplasia, suggesting that the smaller of paired arteries are more vulnerable to occlusion. Existing cohort studies further support clinical observations that hypoplastic vertebral artery enhances synergistically the vascular risk for posterior circulation ischemic events and is closely associated with both atherosclerotic and prothrombotic processes.

  13. Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis.

    Science.gov (United States)

    Solanki, Shailesh; Babu, M Narendra; Gowrishankar; Ramesh, S

    2016-01-01

    A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis. PMID:27274132

  14. Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis

    Directory of Open Access Journals (Sweden)

    Shailesh Solanki

    2016-01-01

    Full Text Available A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis.

  15. Lissencephaly-pachygyria and cerebellar hypoplasia in a calf

    OpenAIRE

    Bianca Lemos dos Santos; Maria Cecília Florisbal Damé; Ana Carolina Barreto Coelho; Plínio Aguiar de Oliveira; Clairton Marcolongo-Pereira; Ana Lucia Schild

    2016-01-01

    ABSTRACT: A case of lissencephaly-pachygyria and cerebellar hypoplasia diagnosed in a Charolais x Tabapuã calf is described. The calf presented since birth, clinical signs characterized by apathy, prolonged recumbency, tremors of the head and neck, ataxia, hypermetria, difficulty walking, blindness and swelling of the joints of the four limbs. Due to the unfavorable prognosis, the animal was euthanized and necropsied at 34 days of age. At necropsy, a rudimentary development of the brain folds...

  16. Focal dermal hypoplasia: ultrastructural abnormalities of the connective tissue.

    Science.gov (United States)

    del Carmen Boente, María; Asial, Raúl A; Winik, Beatriz C

    2007-02-01

    We followed over 10 years three girls with focal dermal hypoplasia syndrome. The histopathological changes demonstrated at the optical level an hypoplastic dermis with thin and scarce collagen bundles and a marked diminution of elastic fibers. Mature adipose tissue was found scattered within the papillary and reticular dermis. No alterations in the basal membrane were observed by immunocytochemical or ultrastructural techniques. Ultrastructurally, in the skin-affected areas, loosely arranged collagen bundles composed of few fibrils were seen scattered in the extracellular matrix. Scarce elastic fibers of normal morphology were also observed. Fibroblasts were smaller, oval-shaped, and diminished in number with a poorly developed cytoplasm. In these fibroblasts, the most conspicuous feature was a remarkable and irregular thickening of the nuclear fibrous lamina. Taking into account that a common link between all laminopaties may be a failure of stem cells to regenerate mesenchymal tissue, this failure would induce the dermal hypoplasia observed in our patients presenting Goltz syndrome.

  17. Aesthetic approach for anterior teeth with enamel hypoplasia

    Directory of Open Access Journals (Sweden)

    Josué Martos

    2012-01-01

    Full Text Available Enamel hypoplasia is a developmental defect of the enamel that is produced by a disturbance in the formation of the organic enamel matrix, clinically visible as enamel defects. Disorders that occur during the stages of enamel development and maturation reduce the amount or thickness of the enamel, resulting in white spots, tiny grooves, depressions and fissures in the enamel surface. The complexity and intensity of the dental deformity lesions will conduct the ideal treatment-associating conservative techniques. This article presents a case report of a restorative treatment of enamel hypoplasia using hybrid composite resin to mask color alteration and enamel defects. An aesthetic appearance that respects the tooth polychromatic and the self-esteem of the patient can be achieved with this approach.

  18. Blaschko Linear Enamel Defects - A Marker for Focal Dermal Hypoplasia: Case Report of Focal Dermal Hypoplasia

    Directory of Open Access Journals (Sweden)

    Stefan Gysin

    2015-05-01

    Full Text Available Focal dermal hypoplasia (FDH is a rare genetic skin disorder. The inheritance of FDH or Goltz-Gorlin syndrome is X-linked dominant and the disease is associated with a PORCN gene mutation. This gene plays a key role in the Wnt pathway, which has an impact on embryonic development. Every tissue derived from meso- and ectoderm can be affected. Patients suffer from cutaneous, ocular, osseous, oral and dental defects. The skin and dental alterations manifest along the Blaschko lines. We present a woman (born in 1962 suffering from FDH with congenital skin changes and Blaschko linear enamel defects. Typical symptoms (e.g. fat herniations, scoliosis, syndactyly, microphthalmia, caries and alopecia plus vertical grooving of all teeth gave a first indication. Molecular genetic testing confirmed the definitive diagnosis of FDH. We hypothesize that, in the context of typical skin changes, visible Blaschko lines on the teeth in the form of vertical grooves are almost pathognomonic for FDH.

  19. Novel PORCN mutations in focal dermal hypoplasia.

    Science.gov (United States)

    Froyen, G; Govaerts, K; Van Esch, H; Verbeeck, J; Tuomi, M-L; Heikkilä, H; Torniainen, S; Devriendt, K; Fryns, J-P; Marynen, P; Järvelä, I; Ala-Mello, S

    2009-12-01

    Focal dermal hypoplasia (FDH), Goltz or Goltz-Gorlin syndrome, is an X-linked dominant multisystem disorder characterized primarily by involvement of the skin, skeletal system and eyes. We screened for mutations in the PORCN gene in eight patients of Belgian and Finnish origin with firm clinical suspicion of FDH. First, we performed quantitative PCR (qPCR) analysis to define the copy number at this locus. Next, we sequenced the coding regions and flanking intronic sequences of the PORCN gene. Three de novo mutations were identified in our patients with FDH: a 150-kb deletion removing six genes including PORCN, as defined by qPCR and X-array-CGH, and two heterozygous missense mutations; c.992T>G (p.L331R) in exon 11 and c.1094G>A (p.R365Q) in exon 13 of the gene. Both point mutations changed highly conserved amino acids and were not found in 300 control X chromosomes. The three patients in whom mutations were identified all present with characteristic dermal findings together with limb manifestations, which were not seen in our mutation-negative patients. The clinical characteristics of our patients with PORCN mutations were compared with the previously reported mutation-positive cases. In this report, we summarize the literature on PORCN mutations and associated phenotypes.

  20. Hypoplasia of exocrine pancreas with myocardial necrosis

    Institute of Scientific and Technical Information of China (English)

    Xiang Sheng Zhang; Xin Xiu Xu; Yan Zhang; Sbu Hua Wu

    2000-01-01

    AIM To study the clinical and pathological features of hypoplasia of exocrine pancreas with myocardialnecrosis.METHODS One ease of hypoplasia of exocrine pancreas with myocardial necrosis was autopsied. Theclinical signs and pathological changes were analyzed.RESULTS A 15-month-old boy with hypoplasia of exocrine pancreas was reported. The main clinicalfeatures were steatorrhea and marked underdevelopment. He died of acute heart failure afterhospitalization. Autopsy showed that there were aplasia of exocrine portion and fatty metaplasia ofpancreas, the myocardium revealed focal necrosis and sear formation.CONCLUSION Atrophy of exocrine pancreas and myocardial necrosis exist at the same time, suggestingthat there may be some relationship between them. It was likely that the damaged pancreatic tissue releasedsome active materials that may harm the myocardium or decrease pancreatic juice that results in lack ofnutrient and myocardial necrosis.

  1. Clinical, neuroradiological and genetic findings in pontocerebellar hypoplasia

    NARCIS (Netherlands)

    Namavar, Yasmin; Barth, Peter G; Kasher, Paul R; van Ruissen, Fred; Brockmann, Knut; Bernert, Günther; Writzl, Karin; Ventura, Karen; Cheng, Edith Y; Ferriero, Donna M; Basel-Vanagaite, Lina; Eggens, Veerle R C; Krägeloh-Mann, Ingeborg; De Meirleir, Linda; King, Mary; Graham, John M; von Moers, Arpad; Knoers, Nine; Sztriha, Laszlo; Korinthenberg, Rudolf; Dobyns, William B; Baas, Frank; Poll-The, Bwee Tien; Sival, Deborah

    2011-01-01

    Pontocerebellar hypoplasia is a group of autosomal recessive neurodegenerative disorders with prenatal onset. The common characteristics are cerebellar hypoplasia with variable atrophy of the cerebellum and the ventral pons. Supratentorial involvement is reflected by variable neocortical atrophy, ve

  2. An extensive DeBakey type IIIb aortic dissection with massive right pleural effusion presenting as abdominal pain and acute anemia:particular case report

    Institute of Scientific and Technical Information of China (English)

    Hui-Chun YU; Zhen-Qing WANG; Yuan-Yuan HAO; Feng-Ping AN; Yu-Chuan HU; Rui-Bing DENG; Peng YU; Guang-Bin CUI; He LI

    2015-01-01

    We describe the case of a 79-year-old male presented with sudden onset of abdominal pain and mild breathlessness, and complicated acute progressive anemia with haemoglobin which declined from 120 g/L to 70 g/L within five days. An urgent computed tomography an-giography showed acute thoracic aortic dissection, DeBakey type IIIb, a dissecting aneurysm in the proximal descending thoracic aorta start-ing immediately after the origin of the left subclavian artery and extending distally below the renal arteries with evidence of rupture into the right pleural cavity for massive pleural effusion. Plasma D-dimer, brain natriuretic peptide and C reactive protein level were elevated. Our case showed that D-dimer can be used as a‘rule-out’ test in patients with suspected aortic dissection. A raised BNP may exert a protective role through anti-inflammatory endothelial actions in the systemic circulation.

  3. THE SYNDROME OF AUTOSOMAL RECESSIVE PONTOCEREBELLAR HYPOPLASIA, MICROCEPHALY, AND EXTRAPYRAMIDAL DYSKINESIA (PONTOCEREBELLAR HYPOPLASIA TYPE-2) - COMPILED DATA FROM 10 PEDIGREES

    NARCIS (Netherlands)

    BARTH, PG; BLENNOW, G; LENARD, HG; BEGEER, JH; VANDERKLEY, JM; HANEFELD, F; PETERS, ACB; Valk, J.

    1995-01-01

    The syndrome of autosomal recessive pontocerebellar hypoplasia, microcephaly, severely impaired mental and motor development, and extrapyramidal dyskinesia is a distinct system degeneration, previously designated pontocerebellar hypoplasia type 2 (PCH-2). To further characterize its clinical and neu

  4. MR assessment of fetal pulmonary hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kuwashima, Shigeko; Kohno, Atsushi; Saiki, Natoru; Iimura, Fumitoshi; Kohno, Tatsuo; Hashimoto, Teisuke; Fujioka, Mutsuhisa [Dokkyo Univ. School of Medicine, Mibu, Tochigi (Japan)

    2000-08-01

    The purpose of this study is to evaluate pulmonary hypoplasia of the fetus using MRI. The subjects consisted of 36 fetuses (18 to 40 weeks' gestation). All fetuses or mothers had major anomalies diagnosed on fetal ultrasonography. MR imaging was performed with a 1.5-T magnet and HASTE (half-Fourier acquisition single-shot turbo spin-echo) sequence. MR images were evaluated with special attention to the intensity of the lung. A diagnosis of pulmonary hypoplasia was based on the clinical, surgical, and autopsy findings. All fetuses with normal pulmonary development showed high intensity in the lung, while all fetuses with pulmonary hypoplasia showed a low intensity in the lung, obscured pulmonary vessels and a small thorax. There was a close correlation between the lung intensity and pulmonary growth. MR assessment of lung intensity may facilitate the diagnosis of pulmonary hypoplasia, particularly after 26 weeks' gestation. Some of the normally developing lung showed a low intensity from 20 to 24 weeks of gestational age. The change to normal lung intensity may occur during this period. (author)

  5. Aortic Aneurysm

    Science.gov (United States)

    ... Most aneurysms are in the aorta, the main artery that runs from the heart through the chest and abdomen. There are two types of aortic aneurysm: Thoracic aortic aneurysms (TAA) - these occur in the part of the aorta running through the chest Abdominal aortic aneurysms (AAA) - these occur in the part ...

  6. Enamel hypoplasia: challenges of esthetic restorative treatment.

    Science.gov (United States)

    Ruschel, Vanessa Carla; Araújo, Élito; Bernardon, Jussara Karina; Lopes, Guilherme Carpena

    2016-01-01

    Enamel defects, such as white or yellow-brown spots, usually cause problems that are more esthetic than functional. Enamel hypoplasia may be the result of hereditary, systemic, or local factors. Dental trauma is a local etiologic factor. It is relatively common in the primary dentition and can cause defects on the surface of permanent successors. Treatment for such defects can differ, depending on the depth of the spots. For deeper white-spot lesions, a composite resin restoration may be necessary. This is an excellent mode of treatment, due to both its low cost and its conservation of healthy tooth structure. The objective of this case report is to describe composite resin restoration of a maxillary central incisor affected by enamel hypoplasia.

  7. Enamel hypoplasia: challenges of esthetic restorative treatment.

    Science.gov (United States)

    Ruschel, Vanessa Carla; Araújo, Élito; Bernardon, Jussara Karina; Lopes, Guilherme Carpena

    2016-01-01

    Enamel defects, such as white or yellow-brown spots, usually cause problems that are more esthetic than functional. Enamel hypoplasia may be the result of hereditary, systemic, or local factors. Dental trauma is a local etiologic factor. It is relatively common in the primary dentition and can cause defects on the surface of permanent successors. Treatment for such defects can differ, depending on the depth of the spots. For deeper white-spot lesions, a composite resin restoration may be necessary. This is an excellent mode of treatment, due to both its low cost and its conservation of healthy tooth structure. The objective of this case report is to describe composite resin restoration of a maxillary central incisor affected by enamel hypoplasia. PMID:27599287

  8. Radicular Dentin Dysplasia Associated with Enamel Hypoplasia

    International Nuclear Information System (INIS)

    The author observed a rare case of radicular dentin dysplasia associated with enamel hypoplasia in a 11-year-old boy with a complaint of gum boil formation. 1. Clinically, yellowish-brown colored teeth with severe attrition and several gum boils were observed. 2. Radiographically, obliteration of pulp chamber and root canal, multiple periapical radiolucencies without obvious cause and blunt roots were observed. 3. Systemically, scalp hair and eyebrows were loose and short. And saddle nose could be also seen.

  9. [Oligomeganephronic renal hypoplasia complicated by glomerulonephritis].

    Science.gov (United States)

    Kan'shina, N F; Rykov, V A; Lakhno, P A

    1990-01-01

    Clinico-anatomical data of a rare condition congenital oligomeganephronic renal hypoplasia with a glomerulonephritis as a complication are available for a 13-year-old girl who died of chronic renal failure. Large aglomerular zones consisting of primitive canaliculi in a loose stroma were observed in kidneys that were decreased in size. The glomeruli were few in number, some of them of a large size (2-2.5-fold), firmly attached to the capsule, with pronounced extracapillary proliferation.

  10. Enamel hypoplasia and its role in identification of individuals: A review of literature.

    Science.gov (United States)

    Kanchan, Tanuj; Machado, Meghna; Rao, Ashwin; Krishan, Kewal; Garg, Arun K

    2015-01-01

    Identification of individuals is the mainstay of any forensic investigation especially in cases of mass disasters when mutilated remains are brought for examination. Dental examination helps in establishing the identity of an individual and thus, has played a vital role in forensic investigation process since long. In this regard, description on the role of enamel hypoplasia is limited in the literature. The present article reviews the literature on the enamel hypoplasia and discusses its utility in forensic identification. Enamel hypoplasia is a surface defect of the tooth crown caused by disturbance of enamel matrix secretion. Enamel defects can be congenital or acquired. In cases of mass disasters, or when the body is completely charred, putrefied and mutilated beyond recognition, the unique dental features can help in identification of the victims. PMID:26097340

  11. Bilateral coronoid hypoplasia and complex odontoma: a rare concurrence of developmental pathology and odontogenic tumour of the mandible.

    Science.gov (United States)

    Dar, Mohd Arif; Alaparthi, Ravikiran; Yalamanchili, Samatha; Santosh, Arvind Babu Rajendra

    2015-10-13

    We present a rare case of concurrent bilateral coronoid hypoplasia and complex odontoma in the mandible, with replacement of missing posterior teeth in both sides of the lower jaw. A 20-year-old woman was diagnosed with bilateral occurrence of coronoid hypoplasia and unerupted complex odontoma after radiographic and histopathological examination. The patient was surgically treated with complete removal of the unerupted complex odontoma and prosthetic replacement of the missing teeth.

  12. X-linked congenital adrenal hypoplasia associated with hypospadias in an Egyptian baby: a case report

    Directory of Open Access Journals (Sweden)

    Metwalley Kotb

    2012-12-01

    Full Text Available Abstract Introduction X-linked congenital adrenal hypoplasia is a rare developmental disorder of the human adrenal cortex and is caused by deletion or mutation of the dosage-sensitive sex reversal adrenal hypoplasia congenita critical region of the X chromosome, gene 1 (DAX-1 gene. Most affected children present with failure to thrive, salt wasting and hypoglycemic convulsions in the first months of life. Hypospadias affects approximately one in 250 live male births. Mutations in the mastermind-like domain-containing 1 (MAMLD1 gene have been implicated as one of the causes of hypospadias in children. To the best of our knowledge, an association between congenital adrenal hypoplasia due to a DAX-1 mutation and hypospadias due to mutation of the MAMLD1 gene has not previously been reported in the literature. Case presentation A 35-day-old male Egyptian baby was referred to our institution for the evaluation of a two-week history of recurrent vomiting associated with electrolyte imbalance. On examination, our patient was found to have hypotension and dehydration. A genital examination showed distal penile hypospadias with chordee and normal testes. He had hyponatremia, hyperkalemia, hypoglycemia and metabolic acidosis. Endocrinological investigations revealed low levels of cortisol, 17-hydroxyprogesterone and aldosterone, with a high level of adrenocorticotrophic hormone. A provisional diagnosis of congenital adrenal hypoplasia associated with hypospadias was made. A molecular genetics study confirmed the diagnosis of X-linked congenital adrenal hypoplasia due to DAX-1 mutations and hypospadias due to MAMLD1 mutation. He was started on hydrocortisone and fludrocortisone treatment. After three weeks of treatment, his symptoms improved and his blood sugar, sodium, potassium and cortisol levels normalized. Conclusions We report the case of an Egyptian baby with an association of congenital adrenal hypoplasia due to DAX-1 mutation and hypospadias due

  13. [Inflammatory abdominal aortic aneurysm].

    Science.gov (United States)

    Ziaja, K; Sedlak, L; Urbanek, T; Kostyra, J; Ludyga, T

    2000-01-01

    The reported incidence of inflammatory abdominal aortic aneurysm (IAAA) is from 2% to 14% of patients with abdominal aortic aneurysm and the etiology of this disease is still discussed--according to the literature several pathogenic theories have been proposed. From 1992 to 1997 32 patients with IAAA were operated on. The patients were mostly symptomatic--abdominal pain was present in 68.75% cases, back pain in 31.25%, fever in 12.5% and weight loss in 6.25% of the operated patients. In all the patients ultrasound examination was performed, in 4 patients CT and in 3 cases urography. All the patients were operated on and characteristic signs of inflammatory abdominal aortic aneurysm like: thickened aortic wall, perianeurysmal infiltration or retroperitoneal fibrosis with involvement of retroperitoneal structures were found. In all cases surgery was performed using transperitoneal approach; in three cases intraoperatively contiguous abdominal organs were injured, which was connected with their involvement into periaortic inflammation. In 4 cases clamping of the aorta was done at the level of the diaphragmatic hiatus. 3 patients (9.37%) died (one patient with ruptured abdominal aortic aneurysm). Authors present diagnostic procedures and the differences in the surgical tactic, emphasizing the necessity of the surgical therapy in patients with inflammatory abdominal aortic aneurysm.

  14. Pontoneocerebellar hypoplasia: definition of MR features

    International Nuclear Information System (INIS)

    We report five patients with pontoneocerebellar hypoplasia. Because the issue of cerebellar malformations is a difficult subject, we tried to define criteria for diagnosis on MRI: a thin flat pons with disappearance of the anterior curve, a small cerebellum with predominant flattening of the hemispheres and shortened cerebellar fissures, in contrast to atrophy. The posterior fossa is not enlarged. We emphasize the probable late onset of the disease in fetal life because of the demonstration of the abnormalities at US during the last trimester of the pregnancy in one patient. Prenatal diagnosis is important because of possible autosomal recessive transmission. (orig.)

  15. Arterial Stiffness Alterations and Inflammatory Response Following Endovascular Aortic Repair: Based on a Presentation at the 2013 VEITH Symposium, November 19-23, 2013 (New York, NY, USA).

    Science.gov (United States)

    Moulakakis, Konstantinos G; Mylonas, Spyridon N; Kakisis, John; Kadoglou, Nikolaos P E; Papadakis, Ioannis; Sfyroeras, George S; Antonopoulos, Constantine C N; Mantas, George; Ikonomidis, Ignatios; Liapis, Christos D

    2015-04-01

    Endovascular abdominal aortic aneurysm repair (EVAR) and thoracic aortic aneurysm repair (TEVAR) have been widely incorporated into clinical practice. However, changes in arterial stiffness and post-implantation syndrome after aortic endografting remain important issues under investigation. The aneurysm sac wall motion after successful EVAR and TEVAR reflects complex interactions between all the components of the excluded aneurysm, including true compliance of the aneurysm wall itself, intra-aneurysm sac pressure, remodeling of the thrombus, and mechanical characteristics of the endograft. Experimental and clinical studies have shown that aortic endografting results in increased arterial stiffness in animal models. It can be assumed that the alterations of aortic mechanical properties can have a direct impact on heart output. The long-term impact of these mechanical changes on cardiovascular outcomes and the potential effects of different endografts on hemodynamics are important issues under investigation. Post-implantation syndrome (PIS) is a systemic inflammatory response frequently observed after endovascular treatment of aortic pathologies. The main features of PIS include fever, leukocytosis, elevated C-reactive protein levels, and coagulation disturbances. Endograft design appears to influence this inflammatory response following aortic endografting; woven polyester endografts have been shown to be associated with greater inflammatory response compared to PTFE stent grafts. The purpose of this paper is to review the literature to elucidate arterial stiffness alterations and inflammatory response after EVAR and TEVAR and the impact of endograft design on aortic stiffness and the post-inflammatory response. PMID:26798761

  16. Esophageal Atresia and Tracheoesophageal Fistula with Unilateral Pulmonary Agenesis - Hypoplasia

    Directory of Open Access Journals (Sweden)

    Katragadda Laxmi Narsimha Rao

    2013-04-01

    Full Text Available Association of unilateral severe pulmonary hypoplasia or agenesis and esophageal atresia (EA with or without tracheoesophageal fistula (TEF is an exceedingly rare and highly lethal combination. We report a case of full term male baby who had EA with TEF and right lung hypoplasia, managed at our centre. He is alive and doing well at 10 years of age.

  17. Classification, diagnosis and potential mechanisms in pontocerebellar hypoplasia

    NARCIS (Netherlands)

    Y. Namavar; P.G. Barth; B.T. Poll-The; F. Baas

    2011-01-01

    Pontocerebellar Hypoplasia (PCH) is group of very rare, inherited progressive neurodegenerative disorders with prenatal onset. Up to now seven different subtypes have been reported (PCH1-7). The incidence of each subtype is unknown. All subtypes share common characteristics, including hypoplasia/atr

  18. The present studies of the role of the aortic vascular smooth muscle apoptosis and Hippo-YAP signaling pathway in the development of aortic dissection%主动脉血管平滑肌细胞凋亡和Hippo-YAP信号通路作用于主动脉夹层发病的研究进展

    Institute of Scientific and Technical Information of China (English)

    姜文剑; 兰峰; 张宏家

    2016-01-01

    Aortic dissection is a kind of fatal cardiovascular disease, and the apoptosis of aortic vascular smooth muscle plays an important role in aortic dissection.The new discovered Hippo-YAP signal transduction pathway is significant in regulation of the function of vascular smooth muscle, and it can cause many cardiovascular diseases.This paper aims to review the present studies of the mechanism of the apoptosis of aortic vascular smooth muscle and Hippo-YAP signaling pathway in the pathogenesis of cardiovascular diseases, especially the aortic dissection.%主动脉夹层是一种致命的心血管疾病,主动脉血管平滑肌的凋亡是造成主动脉夹层发生的重要原因.而新近发现的Hippo-YAP信号传导通路在血管平滑肌的功能调节中发挥重要作用,进而造成许多心血管疾病的发生.本文旨在回顾现有的关于主动脉血管平滑肌凋亡和Hippo-YAP信号传导通路在心血管疾病,特别是主动脉夹层发病中作用机制的研究.

  19. Aortic stenting.

    Science.gov (United States)

    Droc, Ionel; Calinescu, Francisca Blanca; Droc, Gabriela; Blaj, Catalin; Dammrau, Rolf

    2015-01-01

    The approach to aortic pathology is nowadays more and more endovascular at both thoracic and abdominal levels. Thoracic stenting has gained worldwide acceptance as first intention to treat pathologies of the descending thoracic aorta. Indications have been extended to aortic arch aneurysms and also to diseases of the ascending aorta. The current devices in use for thoracic endovascular repair (TEVAR) are Medtronic Valiant, Gore TAG, Cook Tx2 and Jotec. The choice of the endograft depends on the thoracic aortic pathology and the anatomical suitability. The technological evolution of the abdominal aortic endografts was very rapid, arriving now at the fourth generation. We report the results of 55 elective cases of endovascular abdominal aortic repair (EVAR) performed in two vascular surgical centers in Romania and Germany. The prostheses used were 16 E-vita Abdominal XT, 12 Excluder, eight Talent, seven PowerLink, three Endurant and nine custom-made, fenestrated or branched from Jotec. The mean follow-up was 18 months with CT-scan, duplex ultrasound and contrast-enhanced ultrasound. The mortality was 2%. EVAR tends to become the gold standard for abdominal aortic aneurysm repair. Technological development of the devices with lowest profile introduction systems will permit to extend the anatomical indications to new frontiers. PMID:26200430

  20. Artery Agenesis: Ipsilateral Common Carotid Artery Hypoplasia

    Directory of Open Access Journals (Sweden)

    Omer Kaya

    2014-01-01

    Full Text Available A 42-year-old female patient, who had been diagnosed with an occlusion of her left internal carotid artery (ICA following Doppler ultrasonographic (US and digitally-subtracted angiographic (DSA examinations performed in an outer healthcare center in order to eliminate the underlying cause of her complaint of amorosis fugax, later applied to our hospital with the same complaint. At Doppler US performed in our hospital’s radiology department, her right common carotid artery (CCA was normal, but her left CCA was hypoplastic. The right internal artery (ICA was validated as normal. At the left side, however, the ICA was apparent only as a stump and it did not demonstrate a continuity. The diagnosis of ICA agenesis was confirmed by the utilization of Doppler US, CT, and DSA imaging, and it was concluded also that ipsilateral CCA hypoplasia could be evaluated as an important clue to the diagnosis of ICA agenesis.

  1. [Aortic aneurysm].

    Science.gov (United States)

    Villar, Fernando; Pedro-Botet, Juan; Vila, Ramón; Lahoz, Carlos

    2013-01-01

    Aortic aneurysm is one important cause of death in our country. The prevalence of abdominal aortic aneurism (AAA) is around 5% for men older than 50 years of age. Some factors are associated with increased risk for AAA: age, hypertension, hypercholesterolemia, cardiovascular disease and, in particular, smoking. The medical management of patients with an AAA includes cardiovascular risk treatment, particularly smoking cessation. Most of major societies guidelines recommend ultrasonography screening for AAA in men aged 65 to 75 years who have ever smoked because it leads to decreased AAA-specific mortality. PMID:24238836

  2. Quadricuspid aortic valve with ruptured sinus of Valsalva.

    Science.gov (United States)

    Akerem Khan, Shamruz Khan; Tamin, Syahidah Syed; Burkhart, Harold M; Araoz, Philip A; Young, Phillip M

    2013-02-01

    We present a case of a 24-year-old woman who was diagnosed with quadricuspid aortic valve with ruptured sinus of Valsalva. Quadricuspid aortic valve is a rare congenital cardiac anomaly. The recognition of quadricuspid aortic valve has clinical significance as it causes aortic valve dysfunction, and is often associated with other congenital cardiac abnormalities. We showed the important role of multimodality imaging in diagnosing a quadricuspid aortic valve associated with ruptured sinus of Valsalva. PMID:22874066

  3. Enamel hypoplasia in the middle pleistocene hominids from Atapuerca (Spain).

    Science.gov (United States)

    Bermúdez de Castro, J M; Pérez, P J

    1995-03-01

    The prevalence and chronology of enamel hypoplasias were studied in a hominid dental sample from the Sima de los Huesos (SH) Middle Pleistocene site at the Sierra de Atapuerca (Burgos, northern Spain). A total of 89 permanent maxillary teeth, 143 permanent mandibular teeth, and one deciduous lower canine, belonging to a minimum of 29 individuals, were examined. Excluding the antimeres (16 maxillary and 37 mandibular cases) from the sample, the prevalence of hypoplasias in the permanent dentition is 12.8% (23/179), whereas the deciduous tooth also showed an enamel defect. No statistically significant differences were found between both arcades and between the anterior and postcanine teeth for the prevalence of hypoplasias. In both the maxilla and the mandible the highest frequency of enamel hypoplasias was recorded in the canines. Only one tooth (a permanent upper canine) showed two different enamel defects, and most of the hypoplasias were expressed as faint linear horizontal defects. Taking into account the limitations that the incompleteness of virtually all permanent dentitions imposes, we have estimated that the frequency by individual in the SH hominid sample was not greater than 40%. Most of the hypoplasias occurred between birth and 7 years (N = 18, X = 3.5, SD = 1.3). Both the prevalence and severity of the hypoplasias of the SH hominid sample are significantly less than those of a large Neandertal sample. Furthermore, prehistoric hunter-gatherers and historic agricultural and industrial populations exhibit a prevalence of hypoplasias generally higher than that of the SH hominids. Implications for the survival strategies and life quality of the SH hominids are also discussed. PMID:7785727

  4. Subdural effusions and lack of early pontocerebellar hypoplasia in siblings with RARS2 mutations.

    Science.gov (United States)

    Kastrissianakis, Katherina; Anand, Geetha; Quaghebeur, Gerardine; Price, Sue; Prabhakar, Prab; Marinova, Jasmina; Brown, Garry; McShane, Tony

    2013-12-01

    Mutations in the recently described RARS2 gene encoding for mitochondrial arginyl-transfer RNA synthetase give rise to a disorder characterised by early onset seizures, progressive microcephaly and developmental delay. The disorder was named pontocerebellar hypoplasia type 6 (PCH6) based on the corresponding radiological findings observed in the original cases. We report two siblings with the RARS2 mutation who displayed typical clinical features of PCH6, but who had distinct neuroimaging features. Early scans showed marked supratentorial, rather than infratentorial, atrophy, and the pons remained preserved throughout. One sibling also had bilateral subdural effusions at presentation. The deceleration in head growth pointed to an evolving genetic/metabolic process giving rise to cerebral atrophy and secondary subdural effusions. RARS2 mutations should be considered in infants presenting with seizures, subdural effusions, decelerating head growth and evidence of cerebral atrophy even in the absence of pontocerebellar hypoplasia on imaging. PMID:24047924

  5. Hemoptise e hemotórax como apresentação de ruptura de aorta torácica Hemoptysis and hemothorax as presentation of thoracic aortic rupture

    Directory of Open Access Journals (Sweden)

    Márcio Silva Miguel Lima

    2009-06-01

    Full Text Available Dissecção da aorta torácica é doença de grande mortalidade em sua fase inicial, mas pode, em alguns casos, se cronificar. Relatamos caso de paciente com dissecção crônica de aorta tipo B (Stanford, admitido na Emergência com confusão mental, dispnéia e relato de hemoptise importante. O eletrocardiograma mostrava alterações inespecíficas e a radiografia de tórax revelou opacificação do hemitórax esquerdo. O ecocardiograma transtorácico não evidenciou dissecção aórtica, mas demonstrou imagem compatível com hemotórax, ocasionando a suspeita de ruptura da aorta. O paciente evoluiu em colapso cardiovascular e óbito. Este caso descreve duas apresentações atípicas da dissecção de aorta: hemotórax e hemoptise importante.Thoracic aortic dissection is a disease of great mortality in its initial phase, but in some cases it can assume chronic course. We report a case of a patient with Stanford type A1 aortic dissection, admitted with mental confusion, dyspnea and event of severe hemoptysis. Electrocardiogram showed unspecific change and chest X-ray revealed opacification of the left hemithorax. Transthoracic echocardiogram did not show aortic dissection, but showed image similar to hemithorax leading to the suspicion of aortic rupture. The patient developed cardiovascular collapse and evolved to death. This case describes two unusual presentations of aortic dissection: hemothorax and severe hemoptysis.

  6. Ischemic stroke in patient with existing congenital hypoplasia of the middle cerebral artery

    International Nuclear Information System (INIS)

    Presented is a clinical case of a woman 29 years old with ischemic stroke (IS), which has developed abruptly in existing congenital hypoplasia and occlusion of the middle cerebral artery. There are no other well or less well documented risk factors for cerebrovascular disease. In family history noted that the father of the patient died suddenly at the age of 45 years from stroke, also without evidence of vascular disease. On magnetic resonance imaging (MRI) of the brain is found high signal zone in the left nucleus lentiformis. We discussed the possibilities for implementing conventional angiography and eventually surgical procedures unfortunately rejected due to the high risk to the patient. Key words: Ischemic Stroke. Magnetic Resonance Imaging. Hypoplasia

  7. The pathogenesis of renal dysplasia. I. Quantification of hypoplasia and dysplasia.

    Science.gov (United States)

    Schwarz, R D; Stephens, F D; Cussen, L J

    1981-09-01

    In order to assess the relative effects of abnormal ureteric orifice position and abnormal urodynamics on the morphogenesis of hypoplasia and dysplasia in kidneys obtained from infants, we devised a method of quantifying the renal structures. The method was based on radial glomerular counts which ranged from zero to normal (seven to nine), a score for dysplastic structures, and the ratio of normal to abnormal tissues present. These three values, when plotted against each other, correlated closely. The glomerular count, with occasional minor adjustment for inconsistencies, was the best parameter of hypodysplasia. Severe to moderate grades of hypodysplasias fell in the low and middle ranges and hypoplasia through to normal in the highest range. By grading kidneys in this way, we were able to compare the effects of ureteral ectopy and abnormal urinary dynamics on the developing kidney. PMID:7024184

  8. Nonsyndromic hemimandibular hypoplasia: A case report with a short review of the literature

    Directory of Open Access Journals (Sweden)

    Sourav Bhattacharya

    2015-01-01

    Full Text Available Mandibular hypoplasia (MH is a condition, which characterizes deviation in the mandibular growth patterns causing functional and esthetic alterations. MHs encountered in the craniofacial region may be of three types: Congenital, developmental and acquired. Majority of the MHs are associated with syndromes with very little emphasis on the "nonsyndromic" category. Furthermore, the hypoplasias generally have a bilateral manifestation on the mandible. We focus on the nonsyndromic category and report a case of isolated unilateral MH in a 17-year-old boy with obvious facial disfigurement but with no association to any syndrome. The objective of this presentation is to highlight the rarity, possible manifestations and diagnostic approach of the nonsyndromic group.

  9. Abnormal fetal movements, micrognathia and pulmonary hypoplasia: a case report. Abnormal fetal movements

    Directory of Open Access Journals (Sweden)

    Morokuma Seiichi

    2010-08-01

    Full Text Available Abstract Background Micrognathia is a facial malformation characterized by mandibular hypoplasia and a small, receding chin that fails to maintain the tongue in a forward position. We previously reported a system of prenatal screening that we developed to identify fetuses with compromised central nervous system function by observing fetal behavior. In this paper we report the case of a preterm infant with micrognathia and pulmonary hypoplasia who presented abnormal fetal movements. Case presentation A 27-year-old Japanese primigravida at 33 weeks of gestation was referred to our hospital. Ultrasonographic examination revealed clinical polyhydramnios. Micrognathia was evident on midsagittal and 3 D scan. The lung area was less than the mean -2.0 standard deviations for the gestational age. The infant had mandibular hypoplasia and glossoptosis. After emergency cesarean delivery for non-reasuring fetal status, required immediate tracheostomy and cardiopulmonary resuscitation with mechanical ventilatory support. However, the infant's cardiopulmonary condition did not improve and she died 21 hours after birth. Conclusions The findings of our ultrasound exam are suggestive of brain dysfunction. The observation of fetal behavior appears to be effective for the prediction of prognosis of cases with micrognathia.

  10. Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism

    Directory of Open Access Journals (Sweden)

    Masuda N

    2014-09-01

    Full Text Available Naonori Masuda, Taiji Hasegawa, Mariko Yamashita, Nahoko Ogata Department of Ophthalmology, Nara Medical University, Nara, Japan Abstract: Oculocutaneous albinism is a group of congenital disorders caused by alterations of melanin biosynthesis. We report our findings in a patient with oculocutaneous albinism who presented with foveal hypoplasia and a foveal hemorrhage. A 48-year-old man noted a dark spot in the middle of the visual field of his right eye. He had depigmented skin, white hair, white eyebrows, and white cilia. He also had horizontal nystagmus and depigmented irides. His best-corrected visual acuity was 2/100 with -14.0 diopters in the right eye and 3/100 with -5.0 diopters in the left eye. Ophthalmoscopy showed diffuse depigmentation in both eyes and a foveal hemorrhage in the right eye. Optical coherence tomography showed the absence of a foveal pit in both eyes and a subretinal hyperreflective lesion corresponding to the foveal hemorrhage in the right eye. Fluorescein angiography showed that the retinal and choroidal vessels were relatively hypofluorescent because of the lack of a blocking effect of the pigments in the retinal pigment epithelium. Fluorescein angiography and indocyanine green angiography did not show any evidence of choroidal neovascularization in either eye. The foveal hemorrhage in the right eye spontaneously regressed and finally resolved at 3 months after onset. At the final examination, the patient reported that his vision had recovered. A foveal hemorrhage is a rare condition in an eye with foveal hypoplasia associated with albinism. The hemorrhage may be related to high myopia and also to the hypoplasia of the fovea associated with albinism. Keywords: albinism, foveal hemorrhage, foveal hypoplasia, simple hemorrhage

  11. Phenotypic and molecular characterization of focal dermal hypoplasia in 18 individuals.

    Science.gov (United States)

    Bostwick, Bret; Fang, Ping; Patel, Ankita; Sutton, V Reid

    2016-03-01

    Focal dermal hypoplasia, or Goltz syndrome, is a highly variable X-linked dominant disorder with abnormalities in ectoderm and mesoderm derived tissues. Classic clinical features include patchy hypoplastic skin, split hand/foot deformities, and ocular malformations. We aimed to refine the understanding of the phenotypic spectrum and natural history of this disorder and now present multi-disciplinary clinical description and medical history review for 18 patients with focal dermal hypoplasia. All disease characteristics were analyzed and compiled in aggregate to aid in development of clinical diagnostic criteria. Medical history data unexpectedly revealed that the majority of patients (87%) had undergone tonsillectomy for obstructive sleep apnea, which exposed an important co-morbidity that is not well described in the literature, but managing physicians should be made aware of. Fifteen of the 18 patients underwent molecular sequencing of PORCN to detect heterozygous or mosaic mutations. Where no mutation was detected, we performed exon-targeted chromosomal microarray to evaluate for large deletions of the PORCN gene region. We detected a pathogenic genotype in 14 of 15 patients, including one novel chromosomal deletion and four novel PORCN sequence variants. Here, we provide phenotypic summary analysis of 18 patients with focal dermal hypoplasia and propose clinical diagnostic criteria. PMID:26853229

  12. Bone marrow hypoplasia in a cat treated with griseofulvin.

    Science.gov (United States)

    Rottman, J B; English, R V; Breitschwerdt, E B; Duncan, D E

    1991-02-01

    Three weeks after initiation of griseofulvin treatment for dermatophytosis (40 mg/kg of body weight, q 12 h), an 8-yr-old domestic shorthair cat developed depression, vomiting, and pyrexia. Abnormalities found during physical examination included bilateral mydriasis, visual impairment, grade-II/V systolic murmur and multiple areas of alopecia. The cat was pancytopenic; serum biochemical abnormalities included hyperbilirubinemia, hyperglycemia, hyponatremia, and hypokalemia, and urinalysis revealed proteinuria, glycosuria, and bilirubinuria. Examination of a bone marrow aspirate revealed profound hypoplasia of all precursors. Griseofulvin toxicosis was diagnosed on the basis of the temporal relationship of drug administration with onset of clinical, hematologic, and biochemical abnormalities and failure to identify an infective or neoplastic cause for the bone marrow hypoplasia. The condition was refractory to treatment and the cat was euthanatized. Pathologic changes in the bone marrow were consistent with severe hypoplasia of all bone marrow precursors.

  13. 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)

  14. Recent developments for surgical aortic valve replacement: the concept of sutureless valve technology

    OpenAIRE

    Carrel, Thierry; Englberger, Lars; Stalder, Mario

    2013-01-01

    Aortic stenosis has become the most frequent type of valvular heart disease in Europe and North America and presents in the large majority of patients as calcified aortic stenosis in adults of advanced age. Surgical aortic valve replacement has been recognized to be the definitive therapy which improves considerably survival for severe aortic stenosis since more than 40 years. In the most recent period, operative mortality of isolated aortic valve replacement for aortic stenosis varies betwee...

  15. Bilateral hypoplasia of the internal carotid arteries with basilar aneurysm

    Energy Technology Data Exchange (ETDEWEB)

    Briganti, F.; Tortora, F.; Elefante, A. [Universita di Napoli Federico II, Dipartimento di Scienze Neurologiche, Cattedra di Neuroradiologia, 80131, Napoli (Italy); Maiuri, F. [Universita di Napoli Federico II, Department of Neurological Sciences, Neurosurgery Service, Napoli (Italy)

    2004-10-01

    We report a rare case of bilateral hypoplasia of the internal carotid arteries with an associated aneurysm of the basilar tip, studied by CT angiography, MR angiography and digital angiography. The patient became symptomatic with an episode of loss of consciousness, likely due to reduced blood perfusion. The other 20 reported cases of bilateral carotid hypoplasia (only four of which with an associated aneurysm) are reviewed. The findings of noninvasive procedures (including narrowing of the carotid canals on CT) may lead to a correct diagnosis before angiography is performed; they may also help to differentiate angiographic narrowing of the hypoplastic internal carotids from the string sign often observed in some acquired conditions. (orig.)

  16. Aortic valve bypass

    DEFF Research Database (Denmark)

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

    2013-01-01

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

  17. Failure to demonstrate Chlamydia pneumoniae in symptomatic abdominal aortic aneurysms by a nested polymerase chain reaction (PCR)

    DEFF Research Database (Denmark)

    Lindholt, Jes Sanddal; Ostergård, L; Henneberg, E W;

    1998-01-01

    To investigate whether Chlamydia pneumoniae is present in symptomatic abdominal aortic aneurysms (AAA).......To investigate whether Chlamydia pneumoniae is present in symptomatic abdominal aortic aneurysms (AAA)....

  18. External aortic wrap for repair of type 1 endoleak

    Directory of Open Access Journals (Sweden)

    Anastasia Dean

    2014-01-01

    CONCLUSION: Provided certain anatomical features are present, an external aortic wrap is a useful and successful option to manage type 1 endoleak in high-risk patients who are unsuitable for aortic clamping.

  19. Pregnancy after aortic root replacement in Loeys-Dietz syndrome: High risk of aortic dissection.

    Science.gov (United States)

    Braverman, Alan C; Moon, Marc R; Geraghty, Patrick; Willing, Marcia; Bach, Christopher; Kouchoukos, Nicholas T

    2016-08-01

    Loeys-Dietz syndrome due to mutations in TGFBR1 and 2 is associated with early and aggressive aortic aneurysm and branch vessel disease. There are reports of uncomplicated pregnancy in this condition, but there is an increased risk of aortic dissection and uterine rupture. Women with underlying aortic root aneurysm are cautioned about the risk of pregnancy-related aortic dissection. Prophylactic aortic root replacement is recommended in women with aortopathy and aortic root dilatation to lessen the risk of pregnancy. There is limited information in the literature about the outcomes of pregnancy after root replacement in Loeys-Dietz syndrome. We present a case series of three women with Loeys-Dietz syndrome who underwent elective aortic root replacement for aneurysm disease and subsequently became pregnant and underwent Cesarean section delivery. Each of these women were treated with beta blockers throughout pregnancy. Surveillance echocardiograms and noncontrast MRA studies during pregnancy remained stable demonstrating no evidence for aortic enlargement. Despite the normal aortic imaging and careful observation, two of the three women suffered acute aortic dissection in the postpartum period. These cases highlight the high risk of pregnancy following aortic root replacement in Loeys-Dietz syndrome. Women with this disorder are recommended to be counseled accordingly. © 2016 Wiley Periodicals, Inc. PMID:27125181

  20. When and how to replace the aortic root in type A aortic dissection.

    Science.gov (United States)

    Leshnower, Bradley G; Chen, Edward P

    2016-07-01

    Management of aortic root pathology during repair of acute type A aortic dissection (TAAD) requires a comprehensive evaluation of the patient's anatomy, demographics, comorbidities and physiologic status at the time of emergent operative intervention. Surgical options include conservative repair of the root (CRR) (with or without replacement of the aortic valve), replacement of the native valve and aortic root using a composite valve-conduit and valve sparing root replacement (VSRR). The primary objective of this review is to provide data for surgeons to aid in their decision-making process regarding management of the aortic root during repair of TAAD. No time or language restrictions were imposed and references of the selected studies were checked for additional relevant citations. Multiple retrospective reviews have demonstrated equivalent operative mortality between aortic root repair and replacement during TAAD. There is a higher incidence of aortic root reintervention with aortic root repair compared to aortic root replacement (ARR). Experienced, high-volume aortic centers have demonstrated the safety of VSRR in young, hemodynamically stable patients presenting with TAAD. In conclusion, aortic root repair can safely be performed in the vast majority of patients with TAAD. Despite the increased surgical complexity, ARR does not increase operative mortality and improves the freedom from root reintervention. VSRR can be performed in highly selected populations of patients with TAAD with durable mid-term valve function. PMID:27563551

  1. Unilateral Glenoid Hypoplasia: A Case Report and Review of the Literature

    OpenAIRE

    Ashish Suryawanshi; Amber Mittal; Snehal Dongre; Neeti Kashyap

    2012-01-01

    Glenoid hypoplasia is a relatively rare alteration that in most cases involves the pectoral girdle in a bilateral and symmetrical manner. In general, glenoid hypoplasia is associated with skeletal changes such as hypoplasia of the humeral head or changes in the morphology of the acromion and of the coracoid. We describe a rare case of unilateral glenoid hypoplasia without instability and not involving humeral head. The patient was managed effectively with nonoperative measures that featured s...

  2. Bicuspid Aortic Valve Disease and Ascending Aortic Aneurysms: Gaps in Knowledge

    Directory of Open Access Journals (Sweden)

    Katie L. Losenno

    2012-01-01

    Full Text Available The bicuspid aortic valve is the most common congenital cardiac anomaly in developed nations. The abnormal bicuspid morphology of the aortic valve results in valvular dysfunction and subsequent hemodynamic derangements. However, the clinical presentation of bicuspid aortic valve disease remains quite heterogeneous with patients presenting from infancy to late adulthood with variable degrees of valvular stenosis and insufficiency and associated abnormalities including aortic coarctation, hypoplastic left heart structures, and ascending aortic dilatation. Emerging evidence suggests that the heterogeneous presentation of bicuspid aortic valve phenotypes may be a more complex matter related to congenital, genetic, and/or connective tissue abnormalities. Optimal management of patients with BAV disease and associated ascending aortic aneurysms often requires a thoughtful approach, carefully assessing various risk factors of the aortic valve and the aorta and discerning individual indications for ongoing surveillance, medical management, and operative intervention. We review current concepts of anatomic classification, pathophysiology, natural history, and clinical management of bicuspid aortic valve disease with associated ascending aortic aneurysms.

  3. Infrarenal Aortic Occlusion

    Science.gov (United States)

    Traverso, L. W.; Baker, J. D.; Dainko, E. A.; Machleder, H. I.

    1978-01-01

    Twenty-eight patients with total occlusion of the infrarenal aorta have been seen at the UCLA Hospitals in the past 11 years. Claudication was the presenting complaint in all but one patient, with one-third having ischemic rest pain. The average age of these patients was 54 years, and their histories revealed a surprising absence of myocardial infarction, stroke, or diabetes, although 40% had essential hypertension. Heavy tobacco use, however, was characteristic of the entire group. Arteriography proved valuable in identifying and characterizing the vascular abnormalities, but posed problems in technique and interpretation. Significant distal arterial disease was detected radiographically in only 21% of these patients. Operative correction of the aortic occlusion was performed on 26 patients, 18 by aortic bypass grafts and eight by aorto-iliac endarterectomy, with one early postoperative death. Although the thrombus extended to the renal artery origins in 77% of the cases, a well-designed technical approach did not require renal artery occlusion. Using serial creatinine determinations, one case of renal insufficiency was detected which was associated with prolonged postoperative hypotension. Although the extent of distal disease was more severe in those who underwent bypass, symptoms of claudication returned earlier and were more prominent in the endarterectomy group. This recurrence of systems was not favorably altered by sympathectomy performed concomitantly with the initial procedure. Even though this condition seems to pose difficult technical obstacles and has a poor prognosis, infrarenal aortic occlusion can be successfully treated by aortic bypass, with favorable long-term results, if particular attention is paid to elements of the preoperative evaluation and the intraoperative technical requirements peculiar to this relatively uncommon disease entity. ImagesFig. 1.Fig. 2.Fig. 3. PMID:646479

  4. Open surgical repair of abdominal aortic aneurysm: Proximal aortic control by endoaortic balloon - A novel approach

    OpenAIRE

    Balakrishnan Soundaravalli; Palaniappan, M.; Rajani Sundar; Chandrasekar, P.

    2012-01-01

    Patients with infrarenal abdominal aortic aneurysm with unfavorable anatomy for endovascular aneurysm repair have to undergo open surgical repair. Open surgery has its own morbidity in terms of proximal clamping and declamping, bleeding and prolonged hospital stay and mortality. We present two such patients with juxtarenal abdominal aortic aneurysm who underwent open surgical repair. The proximal aortic control during open surgical repair of the aneurysm was achieved by endoaortic balloon occ...

  5. Open surgical repair of abdominal aortic aneurysm: Proximal aortic control by endoaortic balloon - A novel approach

    Directory of Open Access Journals (Sweden)

    Balakrishnan Soundaravalli

    2012-01-01

    Full Text Available Patients with infrarenal abdominal aortic aneurysm with unfavorable anatomy for endovascular aneurysm repair have to undergo open surgical repair. Open surgery has its own morbidity in terms of proximal clamping and declamping, bleeding and prolonged hospital stay and mortality. We present two such patients with juxtarenal abdominal aortic aneurysm who underwent open surgical repair. The proximal aortic control during open surgical repair of the aneurysm was achieved by endoaortic balloon occlusion technique.

  6. Hybrid treatment of recurring thoracoabdominal aortic aneurysm concomitant with retrograde type A aortic dissection

    Institute of Scientific and Technical Information of China (English)

    ZHANG Min-hong; GUO Wei; DU Xin; XIONG Jiang

    2010-01-01

    So far, standard therapy of complex thoracoabdominal aortic disease is open surgical repair requiring aortic clamping and replacement of the involved segment.Despite significant improvements, morbidity and mortality of open surgery remain high.I As a result, open surgery is often withheld owing to severe comorbidities of the patients. Endovascular technique has emerged as an alternative for treatment of these diseases in high risk patients,2 and has enlarged the options of treatment of complex aortic diseases. However, an endovascular approach alone is often deemed unsuitable for some complex aortic disorders because of the close proximity of the supraaortic or visceral branches. A hybrid open-endovascular approach has therefore been proposed as a viable alternative. We present here a unique patient with recurring thoracoabdominal aortic aneurysm (TAAA) concomitant with an aortic dissection (AD) treated by a hybrid open-endovascular approach.

  7. Aortic Aneurysm Statistics

    Science.gov (United States)

    ... connective tissue disorders, such as Marfan syndrome and Ehlers-Danlos syndrome, get thoracic aortic aneurysms. Signs and symptoms of thoracic aortic aneurysm can include Sharp, sudden pain in the chest or upper back. Shortness of ...

  8. Aortic aneurysm repair - endovascular

    Science.gov (United States)

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

  9. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... to become you to our live webcast. Today we’re going to repair an abdominal aortic aneurysm ... and together as a team of multidisciplinary physicians, we’re going to repair an abdominal aortic aneurysm ...

  10. Aortic growth rates in chronic aortic dissection

    Energy Technology Data Exchange (ETDEWEB)

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

    2007-09-15

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

  11. Lung hypoplasia and patellar agenesis in Ehlers-Danlos syndrome.

    Science.gov (United States)

    Pradhan, P; Deb, J; Deb, R; Chakrabarti, S

    2009-12-01

    A 22-year-old male patient was admitted with severe cough associated with purulent expectoration, left-sided chest pain and breathlessness. There was a history of recurrent respiratory ailments since childhood. The patient appeared younger than his chronological age. His face and ears were both dysmorphic. Clinically, the patient was diagnosed with Ehlers-Danlos syndrome (EDS). Computed tomography of the thoracic region revealed hypoplasia of the left lung and hyperplasia of the right lung. Both the patellae were absent. However, ultrasonography of his abdomen, echocardiography and other routine blood and urine examination showed no gross abnormalities. Although other respiratory tract abnormalities with EDS are not uncommon, unilateral lung hypoplasia and patellar agenesis in EDS make this case unique. PMID:20087544

  12. Optical coherence tomographic findings in optic nerve hypoplasia

    Directory of Open Access Journals (Sweden)

    Daruchi Moon

    2013-01-01

    Full Text Available We investigated a case of unilateral optic nerve hypoplasia using spectral domain optical coherence tomography (SDOCT. Optical coherence tomography was done on both eyes using 5-line Raster scan for the fovea to analyze the retinal nerve fiber layer thickness, inner retinal layer thickness, outer retinal layer thickness, and optic disc cube scan for the disc. Retinal nerve fiber layer thickness, inner retinal layer thickness, and outer retinal layer thickness were manually measured at 21-points of each five lines, and results were compared between both eyes. Retinal nerve fiber layer thickness and inner retinal layer thickness of optic nerve hypoplasia were significantly thinner than the opposite eye, but there was no significant difference in the thickness of the outer retinal layer between both eyes.

  13. Oral rehabilitation for a patient with oligodontia and maxillary hypoplasia

    OpenAIRE

    Chung, Da-Woon; Vang, Mong-Sook; Park, Sang-Won; Lim, Hyun-Pil; Yang, Hong-So

    2009-01-01

    An 18 year old female with oligodontia and maxillary hypoplasia was treated using an interdisciplinary team approach involving orthodontists, maxillofacial surgeons and prosthodontists. Full mouth one-piece fixed partial dentures were the final restoration. The fixed partial dentures fabricated for the maxilla and mandible using the concept of a shortened dental arch resulted in improved esthetics and the masticatory function. This paper describes the treatment procedures for an oligodontia p...

  14. Organ protection during aortic cross-clamping.

    Science.gov (United States)

    Yeung, Kak Khee; Groeneveld, Menno; Lu, Joyce Ja-Ning; van Diemen, Pepijn; Jongkind, Vincent; Wisselink, Willem

    2016-09-01

    Open surgical repair of an aortic aneurysm requires aortic cross-clamping, resulting in temporary ischemia of all organs and tissues supplied by the aorta distal to the clamp. Major complications of open aneurysm repair due to aortic cross-clamping include renal ischemia-reperfusion injury and postoperative colonic ischemia in case of supra- and infrarenal aortic aneurysm repair. Ischemia-reperfusion injury results in excessive production of reactive oxygen species and in oxidative stress, which can lead to multiple organ failure. Several perioperative protective strategies have been suggested to preserve renal function during aortic cross-clamping, such as pharmacotherapy and therapeutic hypothermia of the kidneys. In this chapter, we will briefly discuss the pathophysiology of ischemia-reperfusion injury and the preventative measures that can be taken to avoid abdominal organ injury. Finally, techniques to minimize the risk of complications during and after open aneurysm repair will be presented. PMID:27650341

  15. Shape of the dilated aorta in children with bicuspid aortic valve

    International Nuclear Information System (INIS)

    The dilated aorta in adults with bicuspid aortic valve has been shown to have different shapes, but it is not known if this occurs in children. This observational study was performed to determine if there are different shapes of the dilated aorta in children with bicuspid aortic valve and their association with age, gender, hemodynamic alterations, and degree of aortic enlargement. One hundred and eighty-seven echocardiograms done on pediatric patients (0 – 18 years) for bicuspid aortic valve, during 2008, were reviewed. Aortic valve morphology, shape/size of the aorta, and pertinent hemodynamic alterations were documented. Aortic dilation was felt to be present when at least one aortic segment had a z-score > 2.0; global aortic enlargement was determined by summing the aortic segment z-scores. The aortic shape was assessed by age, gender, valve morphology, and hemodynamic alterations. Aortic dilation was present in 104/187 patients. The aorta had six different shapes designated from S1 through S6. There was no association between the aortic shape and gender, aortic valve morphology, or hemodynamic abnormalities. S3 was the most common after the age of six years and was associated with the most significant degree of global aortic enlargement. The shape of the dilated aorta in children with bicuspid aortic valve does not occur in a uniform manner and multiple shapes are seen. S2 and S3 are most commonly seen. As aortic dilation becomes more significant, a single shape (S3) becomes the dominant pattern

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

    Institute of Scientific and Technical Information of China (English)

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

    2007-01-01

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

  17. Infected abdominal aortic aneurysm due to Morganella morganii: CT findings.

    Science.gov (United States)

    Kwon, Oh Young; Lee, Jong Seok; Choi, Han Sung; Hong, Hoon Pyo; Ko, Young Gwan

    2011-02-01

    An infected aortic aneurysm, or mycotic aneurysm, is a rare arterial dilatation due to destruction of the infected vessel wall. Common pathogens resulting in an infected aortic aneurysm are Salmonella and Clostridium species, as well as Staphylococcus aureus; Morganella morganii, on the other hand, is very rare. An infected abdominal aortic aneurysm has tendencies to grow rapidly and to rupture. The mortality rate is high in patients undergoing emergent surgical intervention. We report the case of a 65-year-old man who presented with an infected abdominal aortic aneurysm caused by M. morganii. A high index of suspicion and imaging tests are necessary in order to diagnose an infected aortic aneurysm.

  18. Diffuse Supravalvular Aortic Stenosis: Surgical Repair in Adulthood

    Directory of Open Access Journals (Sweden)

    Giovanni Ferlan

    2009-01-01

    Full Text Available We present the case of a 54-year-old woman in which a diffuse congenital supravalvular aortic stenosis (SVAS was associated with a severe aortic valve incompetence and heavy calcification of the aortic annulus. Repair consisted in resection of the ascending aorta, patch augmentation of the hypoplastic aortic root and annulus, placement of a 20 mm Dacron tubular graft (Vascutek, Renfrewshire, UK and aortic valve replacement with a mechanical prosthesis (Sorin, Turin, Italy. Follow-up echocardiography demonstrated normal prosthetic valve function and a postoperative three-dimensional computed tomographic scan showed a normal shape of the reconstructed ascending aorta.

  19. Managing elevated intraocular pressure in a patient with optic nerve hypoplasia

    Directory of Open Access Journals (Sweden)

    Karina Marcovitch

    2009-10-01

    Full Text Available Karina Marcovitch, Joseph SowkaNova Southeastern University, College of Optometry, Davie, FL, USABackground: Optic nerve hypoplasia (ONH is a congenital optic disc anomaly, often manifesting with visual deficits.Case: A 51-year-old woman with a history of bilateral amblyopia was referred for glaucoma evaluation due to elevated intraocular pressure. The patient demonstrated the classic nerve head appearance of bilateral ONH with “double ring sign” and indistinguishable cupping. Despite demonstrating functional and structural measurements consistent with glaucomatous optic neuropathy, it was felt that these deficits were more likely longstanding and secondary to ONH. Additionally, in the absence of any amblyogenic factors, it was also concluded that the patient’s bilateral “amblyopia” was the result of ONH. In that the patient presented with a significant and modifiable risk factor for glaucomatous development and pre-existing retinal nerve fiber layer and visual field defects, the patient was treated with topical glaucoma medications. Conclusion: Diagnosing glaucoma in patients with concurrent anomalies is a clinical conundrum. This report provides a review of ONH with emphasis on the common misdiagnosis of amblyopia in these patients as well as presenting a strategy for diagnosing and managing glaucoma in patients with preexisting, confounding conditions. Keywords: optic nerve hypoplasia, glaucoma, visual field loss, scanning laser polarimetry, threshold automated perimetry

  20. [A case of right pulmonary hypoplasia with congenital diaphragmatic hernia and dextrocardia].

    Science.gov (United States)

    Andou, A; Shimizu, N; Okabe, K; Date, H; Teramoto, S

    1992-10-01

    Chest X-ray of a 28-year-old woman revealed an abnormal shadow in the right lower lung field and dextrocardia, for which detailed investigation was performed. Since the CT number of the tumor shadow corresponded to that of the liver on chest CT, diaphragmatic hernia of the liver was suspected, and was confirmed by MRI and angiography of the abdomen. In addition, the pulmonary artery and vein were hypoplastic, and angiography of the pulmonary artery demonstrated pulmonary hypoplasia. This case was considered to have primary pulmonary hypoplasia, because the dextrocardia was considered to have occurred secondary to pulmonary hypoplasia and the diaphragmatic hernia of the liver was not sufficiently large to cause pulmonary hypoplasia. Pulmonary hypoplasia first diagnosed in adulthood is rare, with a clinical course and roentgenographic appearance differing from those of pulmonary hypoplasia in children.

  1. Repositioning of an Intraventricular Dislocated Aortic Valve during Transcatheter Aortic Valve Implantation

    NARCIS (Netherlands)

    Natour, Ehsan; Douglas, Yvonne L.; Jainandunsing, Jayant S.; Schurer, Remco A. J.; van der Werf, Hendrik W.; van den Heuvel, Ad F. M.

    2014-01-01

    The case is presented of a 75-year-old man referred for transcatheter aortic valve implantation. During the procedure the prosthetic aortic valve became dislocated into the left ventricle shortly after expansion. The subsequent steps taken to reposition the valve using only materials at hand are des

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

    Science.gov (United States)

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

    1994-12-01

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

  3. Chronic hepatitis C infection in a patient with bone marrow hypoplasia

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Chronic hepatitis C virus (HCV) infection is associated with multifarious extra-hepatic manifestations; the most described and discussed being mixed cryoglob- ulinemia which is strongly related to B-cell lympho- proliferative disorders (LPDs). We present a case of chronic HCV infection and mixed cryoglobulinemia, with minimal liver involvement. The case is a 53-year- old patient who was diagnosed as having bone marrow hypoplasia at the age of three. She received several blood transfusions to normalize her haemoglobin. At the age of 31, she was diagnosed with rheumatoid ar- thritis on account of her diffuse joint pain and inflam- mation, elevated rheumatoid factor (RF) and Raynaud's phenomenon. Twenty years later, monoclonal gam- mopathy of IgG Lambda (one year later, changed to IgM Kappa) was detected during a routine examina- tion. A bone marrow biopsy showed hypoplasia, Kappa positive B-lymphocytes and low-grade malignant lym- phoma cells. PCR of the bone marrow aspirate was not contributory. No treatment was initiated owing to her poor bone marrow function and she is under regular follow-up.

  4. Potential risk factors in systemic hypoplasia and dental caries at odontogenesis stage

    Directory of Open Access Journals (Sweden)

    Kovach I.V.

    2013-03-01

    Full Text Available Lesion of hard dental tissue with caries process and lesions not connected with caries present overwhelming majority of dental diseases. Causes leading to damage of enamel integrity and dentin with progressing demineralization are known; they allow to choose justified method of primary prophylaxis. Along with it, etiology and pathogenesis of enamel lesions with demineralization signs remains unexplored. Epidemiologic study of prevalence, incidence and severity of lesion of hard dental tissues in 330 pupils, who constantly live on endemic iodine-defficient territory, of three zones – mountain zone, foothill and valley. For comparison, schoolchildren migrated to this territory 2-3 years after birth, and children – residents of favorable as for iodine level in drinking water were examined, 30 children in each group. It was established, that overwhelming number of schoolchildren of endemic zone suffer from endemic goiter of various severity, which sufficiently impacts on protein-mineral ratio of the organism, bone skeleton and teeth including. It was also established, that residents of three various levels of iodine consumption are prone to development of multiple dental caries and systemic enamel hypoplasia. Children, residents of mountain zone suffer the most, with simultaneous lesion of thyroid gland. The role of endemic goiter in systemic dental enamel hypoplasia and multiple caries, which changes protein matrix, negatively impacts on odontogenesis and mineralization degree is underlined.

  5. [Aplasia and hypoplasia of the optic nerve. Comparison of 2 cases].

    Science.gov (United States)

    Pisaturo, C; Dolci, A; Pessagno, A; Padua, G L; Dassio, D A; De Negri, M

    1996-10-01

    Hypoplasia and aplasia of the optic nerve are congenital anomalies characterized respectively by a marked volume reduction (very small papilla, often identifiable only as a rosy-yellowish area from which the retinal vessels emerge) and by the absence of the optic disk (absence of the nerve and mainly of its vessels) and of the visual functions. These anomalies are often associated with malformations of the central nervous system and of the ocular structures. The defects originate in the embryonal period due to the arrested development of the mesodermal component towards the head of the optic nerve. The aim of this study is to try to interpret the different clinical manifestations of the disease by observing two cases (a female and a male) with a clinical picture of aplasia and hypoplasia of the optic nerve respectively. Both cases presented the following clinical characteristics: developmental defects of the optic nerve, severe microcephaly, positive IgG antibodies against CMV. The association of the optic nerve defect with microcephaly is due to the embryogenic origin of the optic vescicle by prosencephalon. The resulting clinical picture is probably the outcome of an interfering process that the authors have presumed in CMV intrauterine infection, after having excluded the most frequent teratogenic agents (maternal diabetes, tabagism, intake of alcohol ad drugs). Moreover, the different degree of ocular involvement and the different time of onset of the intrauterine pathogenic insult in the two cases could account for the different clinical pictures. PMID:9053727

  6. Unilateral Glenoid Hypoplasia: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ashish Suryawanshi

    2011-01-01

    Full Text Available Glenoid hypoplasia is a relatively rare alteration that in most cases involves the pectoral girdle in a bilateral and symmetrical manner. In general, glenoid hypoplasia is associated with skeletal changes such as hypoplasia of the humeral head or changes in the morphology of the acromion and of the coracoid. We describe a rare case of unilateral glenoid hypoplasia without instability and not involving humeral head. The patient was managed effectively with nonoperative measures that featured specific rehabilitation exercises for the shoulder.

  7. Aortic Valve Sparing in Different Aortic Valve and Aortic Root Conditions.

    Science.gov (United States)

    David, Tirone E

    2016-08-01

    The development of aortic valve-sparing operations (reimplantation of the aortic valve and remodeling of the aortic root) expanded the surgical armamentarium for treating patients with aortic root dilation caused by a variety of disorders. Young adults with aortic root aneurysms associated with genetic syndromes are ideal candidates for reimplantation of the aortic valve, and the long-term results have been excellent. Incompetent bicuspid aortic valves with dilated aortic annuli are also satisfactorily treated with the same type of operation. Older patients with ascending aortic aneurysm and aortic insufficiency secondary to dilated sinotubular junction and a normal aortic annulus can be treated with remodeling of the aortic root or with reimplantation of the aortic valve. The first procedure is simpler, and both procedures are likely equally effective. As with any heart valve-preserving procedure, patient selection and surgical expertise are keys to successful and durable repairs. PMID:27491910

  8. Aortic aneurysm secondary to umbilical artery catheterization

    International Nuclear Information System (INIS)

    A 14-month-girl presented with an asymptomatic posterior mediastinal mass. She had a history of prematurity, umbilical artery catheterization, and sepsis. The diagnosis of aortic aneurysm was made by dynamic computed tomography. The aneurysm was successfully resected. (orig.)

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

    Science.gov (United States)

    Gavorník, Peter; Dukát, Andrej; Gašpar, Ľudovít

    2015-01-01

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

  10. Endovascular resection of the native aortic valve before transcatheter aortic valve implantation: state of the art and review.

    Science.gov (United States)

    Navarra, Emiliano; Mosala Nezhad, Zahra; Bollen, Xavier; Gielen, Charles-Edouard; Mastrobuoni, Stefano; De Kerchove, Laurent; Raucent, Benoit; Astarci, Parla

    2016-09-01

    Transcatheter aortic valve implantation was introduced into clinical practice in 2002 as a rescue approach in patients presenting with symptomatic severe aortic stenosis but not eligible for conventional aortic valve replacement. This technique allows implantation of a balloon expandable bioprosthesis without resection of the native aortic valve. Several complications are described as a consequence of the residual highly calcified valve being squeezed against the aortic wall by the stent of the implant. This can result in deformation of the metal stent and paravalvular leakage, risk of occlusion of the coronary ostia, or central and peripheral embolization of valvular debris. To avoid these complications, many authors suggest the possibility to resect and remove the native aortic valve before transcatheter aortic valve implantation. In this field, different authors have described possible techniques and different sources of energy to resect the calcified valve. In this article, we review the development of these experimental techniques and discuss future prospects in this field. PMID:27032472

  11. Sarcoidosis presenting with severe hypocalcaemia.

    LENUS (Irish Health Repository)

    Saeed, A

    2012-01-31

    Disorders of calcium metabolism, especially hypercalcemia and hypercalceuria, are common in sarcoidosis. They are caused by extra renal unsuppressed production of 1,25 dihydroxy vitamin D at the level of the sarcoid granuloma. Hormonal changes during pregnancy have a physiological synergistic effect on this mechanism, which is primarily parathyroid hormone (PTH) dependant. However, the combination of primary hypoparathyroidism with hypocalcemia and sarcoidosis is rare. Di George syndrome, is a dysmorphic disorder characterised by aplasia\\/hypoplasia of thymus and parathyroid glands in addition to aortic arch anamolies and facial dysmorphia. After commencing appropriate treatment this lady made excellent recovary.

  12. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... opens freely when the heart contracts. On the right, we see a picture of the aortic valve ... the aortic valve because the probe is sitting right behind the aortic valve. Lots of patients on ...

  13. Valvular Aortic Stenosis: A Proteomic Insight

    Directory of Open Access Journals (Sweden)

    Fernando Vivanco

    2010-02-01

    Full Text Available Calcified aortic valve disease is a slowly progressive disorder that ranges from mild valve thickening with no obstruction of blood flow, known as aortic sclerosis, to severe calcification with impaired leaflet motion or aortic stenosis. In the present work we describe a rapid, reproducible and effective method to carry out proteomic analysis of stenotic human valves by conventional 2-DE and 2D-DIGE, minimizing the interference due to high calcium concentrations. Furthermore, the protocol permits the aortic stenosis proteome to be analysed, advancing our knowledge in this area. Summary: Until recently, aortic stenosis (AS was considered a passive process secondary to calcium deposition in the aortic valves. However, it has recently been highlighted that the risk factors associated with the development of calcified AS in the elderly are similar to those of coronary artery disease. Furthermore, degenerative AS shares histological characteristics with atherosclerotic plaques, leading to the suggestion that calcified aortic valve disease is a chronic inflammatory process similar to atherosclerosis. Nevertheless, certain data does not fit with this theory making it necessary to further study this pathology. The aim of this study is to develop an effective protein extraction protocol for aortic stenosis valves such that proteomic analyses can be performed on these structures. In the present work we have defined a rapid, reproducible and effective method to extract proteins and that is compatible with 2-DE, 2D-DIGE and MS techniques. Defining the protein profile of this tissue is an important and challenging task that will help to understand the mechanisms of physiological/pathological processes in aortic stenosis valves.

  14. The distraction osteogenesis in midfacial hypoplasia.

    Science.gov (United States)

    Lucchese, Alessandra; Gherlone, Enrico F; Asperio, Paolo; Baena, Ruggero Rodriguez y

    2014-05-01

    Distraction osteogenesis (DO) can generate new bone in a gap between 2 vascularized bone surfaces in response to application of graduated tensile stress across the bone gap. The authors present the clinical result in a cleft patient with severe maxillary deficiency treated by a rigid external distraction (RED) device. A boy complained of both masticatory and psychological problems because of cleft with severe midfacial retrusion. The treatment aimed to create a well-balanced facial profile, increase maxillary incisal display, create proper overjet and overbite, and align his dentition. By the RED system, the traction is applied to the maxilla through the dentition by an intraoral splint. A complete Le Fort I osteotomy was performed, including pterygomaxillary and septal disjunction, with mobilization. Once osteotomy was completed, the halo portion of the RED device was adjusted for the width of the neurocranium and was rigidly fixed around the head with 2 scalp screws on each side. A well-balanced facial profile and a good alignment of the dentition were obtained. The patients had considerable improvement in his self-esteem. Clinical reports have suggested that maxillary advancements achieved by distraction are more stable than those achieved with orthognathic surgery with a minimal influence on velopharyngeal competence.

  15. Management of Mandibular Hypoplasia Using Distraction Osteogenesis Technique

    Institute of Scientific and Technical Information of China (English)

    陶学金; 樊敏; 凌翔; 陈卫民

    2004-01-01

    Summary: By using distraction osteogenesis technique, 3 cases of mandibular hypoplasia were treated by home-made and German-made jaw distractors: including one patient suffered from bilateral ankylosis of temporo-mandibular joint and 2 patients from deficiency of mandible. The duration of distraction osteogenesis was one month. The bone distractor was removed 3 months after operation. Satisfactory results were obtained in all 3 cases. Distraction osteogenesis can successfully be used in mandibular functional reconstruction and has much more advantages than traditional technique.

  16. Cerebellar and brainstem hypoplasia in a child with a partial monosomy for the short arm of chromosome 5 and partial trisomy for the short arm of chromosome 10

    NARCIS (Netherlands)

    Arts, W F M; Hofstee, Y; Drejer, G F; Beverstock, G C; Oosterwijk, J C

    1995-01-01

    A child with hypoplasia of the cerebellum and brainstem in association with an unbalanced translocation, resulting in a partial deletion of the short arm of chromosome 5 and a partial trisomy of the short arm of chromosome 10, is described. A balanced translocation was present in his mother and mate

  17. Abdominal Aortic Aneurysms: Treatments

    Science.gov (United States)

    ... access catheters Vertebroplasty Women and vascular disease Women's health Social Media Facebook Twitter ... Abdominal Aortic Aneurysms Interventional Radiologists Treat Abdominal Aneurysms Nonsurgically Interventional radiologists are vascular ...

  18. Glucose Suppresses Biological Ferroelectricity in Aortic Elastin

    OpenAIRE

    Liu, Yuanming; WANG, YUNJIE; Chow, Ming-Jay; Chen, Nataly Q.; Ma, Feiyue; Zhang, Yanhang; Li, Jiangyu

    2013-01-01

    Elastin is an intriguing extracellular matrix protein present in all connective tissues of vertebrates, rendering essential elasticity to connective tissues subjected to repeated physiological stresses. Using piezoresponse force microscopy, we show that the polarity of aortic elastin is switchable by an electrical field, which may be associated with the recently discovered biological ferroelectricity in the aorta. More interestingly, it is discovered that the switching in aortic elastin is la...

  19. Medical image of the week: aortic ring

    Directory of Open Access Journals (Sweden)

    Wong C

    2014-10-01

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

  20. A rare cause of recurrent aortic dissection.

    Science.gov (United States)

    Agrawal, Yashwant; Gupta, Vishal

    2016-07-01

    We report the case of a 19-year-old man with a history of Loeys-Dietz syndrome (LDS), which was diagnosed when he had a Stanford type A aortic dissection. He also had multiple aneurysms including ones in the innominate, right common carotid, and right internal mammary arteries. He had had multiple procedures including Bentall's procedure, repeat sternotomy with complete arch and valve replacement, and coil embolization of internal mammary artery aneurysm in the past. His LDS was characterized by gene mutation for transforming growth factor-β receptor 1. He presented to our facility with sudden onset of back pain, radiating to the right shoulder and chest. He was diagnosed with Stanford type B aortic dissection and underwent thoracic aorta endovascular repair for his aortic dissection. This case represents the broad spectrum of pathology associated with LDS where even with regular surveillance and aggressive medical management the patient developed Stanford B aortic dissection. PMID:27358537

  1. Can release of urinary retention trigger abdominal aortic aneurysm rupture?

    Science.gov (United States)

    Luhmann, Andreas; Powell-Bowns, Matilda; Elseedawy, Emad

    2013-04-04

    Only 50% of abdominal aortic aneurysms present with the classic triad of hypotension, back pain and a pulsatile abdominal mass. This variability in symptoms can delay diagnosis and treatment. We present the case of a patient presenting with a unique combination of symptoms suggesting that decompression of urinary retention can lead to abdominal aortic aneurysm rupture.

  2. Presentation

    Directory of Open Access Journals (Sweden)

    Paulo Henrique Freire Vieira

    2013-12-01

    Full Text Available This dossier focuses on one of the essential debate topics today about the territorial dimension of the new development strategies concerned with the worsening of the global socioecological crisis, that is: the challenges related to the activation and integration in networks of localized agri-food systems. For its composition, some contributions presented and debated during the VI International Conference on Localized Agri-food System - The LAFS facing the opportunities and challenges of the new global context have been gathered. The event took place in the city of Florianópolis, from May 21th to 25th of 2013. The event was promoted by the Federal University of Santa Catarina (UFSC and by the Center for the International Cooperation on Agricultural Research for Development (CIRAD. Besides UFSC and CIRAD, EPAGRI, State University of Santa Catarina (UDESC, as well as research institutes and universities from other states (UFMG, IEA/SP, UFS, UFRGS and Mexican and Argentinian partners from the RED SIAL Latino Americana also participated in the organization of lectures, discussion tables and workshops.

  3. Imaging in aortic dissection

    International Nuclear Information System (INIS)

    Aortic dissection (AD) is a catastrophic aortic disease. Imaging techniques play an invaluable role in the diagnostic evaluation and management of patients with AD. Major signs of AD with different imaging modalities are described in this article with a pertinent discussion on guidelines for the optimized approach of imaging study (13 refs.)

  4. Aortic arch malformations

    Energy Technology Data Exchange (ETDEWEB)

    Kellenberger, Christian J. [University Children' s Hospital, Department of Diagnostic Imaging, Zuerich (Switzerland)

    2010-06-15

    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.)

  5. Presentation

    Directory of Open Access Journals (Sweden)

    Isidor Marí Mayans

    2004-04-01

    Full Text Available As was the case at the conference, "Humanities professions in the knowledge society", the Director of Humanities and Philology Studies at the UOC, Isidor Marí, presents this Dossier, and the subsequent virtual debate, with the aim of gaining useful conclusions, with specific repercussions on the organisation of the degree studies and its professional projection, especially at this time, which requires study plans to be redesigned in line with the Bologna process. In the author's opinion, we can only make the right operative decisions when we are able to understand the transformations taking place in the humanistic culture framed by the knowledge society, and to do so, debate has to be opened in which students, graduates, academics, researchers, professionals and analysts can all take part.In this article, Isidor Marí analyses the tensions and contradictions that arise when attempts are made to relate the concepts of the professional world, Humanities and the knowledge society. Firstly, neither are Humanities a profession nor the study of Humanities seen by students or society to be adaptable to the definition of professional profiles. However, this highlights an important paradox, as the culture economy, (and, thus, occupations in the cultural sector, is growing increasingly throughout western societies. Likewise, in terms of the relationship between Humanities and the knowledge society, the author describes and analyses how there currently coexist voices foreseeing the worst alongside those that see information and communications technologies opening the way for an enormously positive transformation in human civilisation and a new cultural era.

  6. Presentation

    Directory of Open Access Journals (Sweden)

    Eduardo Vicente

    2013-06-01

    Full Text Available In the present edition of Significação – Scientific Journal for Audiovisual Culture and in the others to follow something new is brought: the presence of thematic dossiers which are to be organized by invited scholars. The appointed subject for the very first one of them was Radio and the invited scholar, Eduardo Vicente, professor at the Graduate Course in Audiovisual and at the Postgraduate Program in Audiovisual Media and Processes of the School of Communication and Arts of the University of São Paulo (ECA-USP. Entitled Radio Beyond Borders the dossier gathers six articles and the intention of reuniting works on the perspectives of usage of such media as much as on the new possibilities of aesthetical experimenting being build up for it, especially considering the new digital technologies and technological convergences. It also intends to present works with original theoretical approach and original reflections able to reset the way we look at what is today already a centennial media. Having broadened the meaning of “beyond borders”, four foreign authors were invited to join the dossier. This is the first time they are being published in this country and so, in all cases, the articles where either written or translated into Portuguese.The dossier begins with “Radio is dead…Long live to the sound”, which is the transcription of a thought provoking lecture given by Armand Balsebre (Autonomous University of Barcelona – one of the most influential authors in the world on the Radio study field. It addresses the challenges such media is to face so that it can become “a new sound media, in the context of a new soundscape or sound-sphere, for the new listeners”. Andrew Dubber (Birmingham City University regarding the challenges posed by a Digital Era argues for a theoretical approach in radio studies which can consider a Media Ecology. The author understands the form and discourse of radio as a negotiation of affordances and

  7. Acute aortic and mitral valve regurgitation following blunt chest trauma.

    Science.gov (United States)

    Bernabeu, Eduardo; Mestres, Carlos A; Loma-Osorio, Pablo; Josa, Miguel

    2004-03-01

    Traumatic rupture of intracardiac structures is an uncommon phenomenon although there are a number of reports with regards to rupture of the tricuspid, mitral and aortic valves. We report the case of a 25-year-old patient who presented with acute aortic and mitral valve regurgitation of traumatic origin. Both lesions were seen separated by 2 weeks. Pathophysiology is reviewed. The combination of both aortic and mitral lesions following blunt chest trauma is almost exceptional.

  8. Presentation

    Directory of Open Access Journals (Sweden)

    Nicanor Lopes

    2010-11-01

    Full Text Available The Journal Caminhando debuts with a new editorial format: eachmagazine will have a Dossier.In 2010 Christianity celebrated the centenary of Edinburgh. TheWorld Missionary Conference in Edinburgh in 1910 is regarded by manyas missiological watershed in the missionary and ecumenical movement.So the Faculty of Theology of the Methodist Church (FATEO decidedto organize a Wesleyan Week discussing the issue of mission. For anevent of this magnitude FATEO invited the Rev. Dr. Wesley Ariarajah,Methodist pastor and teacher of Sri Lanka with extensive experience inpastoral ministry in local churches and professor of History of Religionsand the New Testament at the Theological College of Lanka, maintainedby the Protestant Churches in Sri Lanka. In 1981 he was invited to jointhe World Council of Churches, where he presided for over ten years theCouncil of Interreligious Dialogue. From 1992 he served as Deputy GeneralSecretary of the WCC.The following texts are not the speeches of the Rev. Dr. WesleyAriarajah, for they will be published separately. Nevertheless, the journaldialogs with the celebrations of the centenary of Edinburgh, parting formthe intriguing theme: "Mission in the 21st century in Brazil". After all, howis it that mission takes place among us in personal, church, and communityactivities?Within the Dossier, as common to the journal, the textos are organizedas follows: Bible, Theology / History and Pastoral Care. Other items thatdo not fit within the Dossier, but, do articulate mission, can be found inthe section Declarations and Documents and Book Reviews.The authors of the Dossier have important considerations in buildinga contemporary missiological concept considering Brazilian reality.Anderson de Oliveira, in the Bible-Section, presents a significantexegeses of Matthew 26.6-13. What does it mean when Jesus is quotedwith the words: "For the poor always ye have with you, but me ye havenot always." Is this declaration challenging the gospels

  9. Delayed aortic regurgitation caused by a right coronary stent protruding into the aorta.

    Science.gov (United States)

    Quintana, Eduard; Mestres, Carlos A; Congiu, Stefano; Josa, Miguel; Cartañá, Ramon

    2009-11-01

    Aortic valve perforation is an extremely rare complication after percutaneous coronary intervention. The case is presented of a 49-year-old male with aortic valve regurgitation secondary to the intra-aortic protrusion of a right coronary stent. The patient had undergone an apparently successful rescue percutaneous transluminal coronary angioplasty with a drug-eluting stent following failed fibrinolysis, but one month later was readmitted for acute pulmonary edema. Further investigations demonstrated new-onset aortic regurgitation. Medical stabilization was achieved and an elective aortic valve replacement and coronary revascularization performed. Intraoperatively, the stent was found to be partially deployed within the aortic lumen, causing perforation to the non-coronary cusp.

  10. Management of Zinner’s Syndrome Associated with Contralateral Seminal Vesicle Hypoplasia: A Case Report

    Directory of Open Access Journals (Sweden)

    Mehdi Kardoust Parizi

    2013-01-01

    Full Text Available A 27-year-old man presented with chronic hematospermia, painful ejaculation, and primary infertility. Physical examination, transrectal ultrasonography, and pelvic magnetic resonance imaging (MRI demonstrated left seminal vesicle cyst, left renal agenesia, and contralateral seminal vesicle hypoplasia. Hormone workup (LH, FSH, prolactin, and testosterone was normal. Sperm analysis showed oligoasthenozoospermia and low ejaculate volume. We performed transurethral resection of the ejaculatory duct (TUR-ED using methylene blue vasography guidance without surgical-related complications. Hematospermia and painful ejaculation completely improved at 2-month followup, and the patient’s wife experienced a missed abortion thereafter. This patient was considered as a rare variant of Zinner’s syndrome and was managed effectively with a less invasive treatment modality (TUR-ED.

  11. Hypodontia and hypoplasia of mandible: A rare defect caused by combination therapy

    Directory of Open Access Journals (Sweden)

    Senthil Kumar

    2013-01-01

    Full Text Available Hypodontia is defined as the developmental absence of one or more tooth and it can present in varying degrees of severity and severe hypodontia has been defined as the absence of six teeth, excluding third molars. Radiation plays an important role in the treatment of head and neck cancer, in spite of its benefits, radiation has several side-effects in the head and neck region. This article highlights a case report, where hypodontia, hypoplasia of mandible, stunded permanent teeth roots, microdontia, inversion of tooth bud were observed in 19 years old patient who was diagnosed with Langerhans cell histiocytosis at her age of 3 years she had received a combination of radiotherapy and chemotherapy for a period of 1 year and radiation of 50 Gy was used in the head and neck region. Radiographs were taken and it revealed abnormal changes in growth and development of bone and teeth.

  12. Rare Case of Unilateral Hypoplasia of Lung with Associated Ventricular Mass in an Adult

    Science.gov (United States)

    Alam, Azad; Iyer, Aparna; Kutty, Jayalakshmi Thelapurath

    2016-01-01

    Hypoplasia of the lung is a rare congenital condition which can be: a) primary i.e. no apparent cause is found; or b) secondary i.e. associated with other congenital anomalies that are implicated in its pathogenesis. These anomalies may involve the diaphragm, cardiovascular, central nervous, urogenital and musculoskeletal system. Patients usually present in neonatal, infancy or childhood period and very rarely in adulthood. Our patient was an adult having a unilateral hypoplastic lung associated with a ventricular mass and to our knowledge this rare combination has never been reported in the English literature; though there are reports of prenatal or newborns with hypoplastic lung and rhabdomyoma of ventricle who did not survive.

  13. Balloon aortic valvuloplasty in pregnancy with severe aortic stenosis and infective endocarditis

    Directory of Open Access Journals (Sweden)

    Vinotha

    2012-12-01

    Full Text Available Twenty seven year old lady, previously diagnosed to have aortic stenosis, presented to the obstetric outpatient department at 19 weeks of gestation with fever and breathlessness, NYHA class 4, for one week. Two D Echo revealed left ventricular hypertrophy, a severely stenosed, calcified bicuspid aortic valve, with vegetations on aortic and mitral valves and severe mitral regurgitation. Blood cultures grew nutrionally variant streptococci and she was treated with crystalline penicillin and gentamicin. She stabilised clinically by 21 weeks, by which time, the risk of termination of pregnancy was comparable to continuing the pregnancy. She underwent balloon aortic valvuloplasty. Post balloon aortic valvuloplasty, she was stable. At 34+2 weeks, she underwent emergency LSCS, the indication being intrauterine growth restriction with fetal compromise and breech presentation. She delivered a baby girl, 1.6 kg, Apgar 9 & 10. Our case report highlights the fact that a timely, balloon aortic valvuloplasty can be life saving for patients with pregnancy complicated by severe aortic stenosis and infective endocarditis. [Int J Reprod Contracept Obstet Gynecol 2012; 1(1.000: 69-71

  14. [Use of sutureless prosthetic aortic valves in cardiac surgery].

    Science.gov (United States)

    Santarpino, Giuseppe; Fischlein, Theodor

    2014-03-01

    In the last years, an increasing proportion of high-risk patients undergo surgical aortic valve replacement. In order to reduce the risk associated with cross-clamp time or cardioplegic ischemic time, sutureless aortic prostheses have been developed. These bioprosthetic valves are not hand sewn, and this technological advance translates into reduced implantation times, thus improving outcome of patients referred for aortic valve replacement. At present, three sutureless bioprostheses are available on the market: 3f Enable (Medtronic Inc., Minneapolis, Minnesota, USA), Perceval (Sorin Group, Saluggia, Italy) and Intuity (Edwards Lifesciences, Irvine, California, USA). This article provides an overview of the available literature on sutureless aortic valves with the aim to better define current role and future perspectives of sutureless aortic bioprostheses for the treatment of aortic valve stenosis. PMID:24770430

  15. Small aortic valve annulus in children with fixed subaortic stenosis.

    Science.gov (United States)

    Thilenius, O G; Campbell, D; Bharati, S; Lev, M; Arcilla, R A

    1989-01-01

    Twenty-one hearts with fixed subaortic stenosis (FSAS) were examined pathologically. Thirty children with no hemodynamically significant heart disease, 31 children with valvar aortic stenosis, and 25 children with FSAS were studied by echo- and angiocardiography. The following conclusions were drawn: (1) Patients with FSAS often have abnormal aortic valve leaflets as well as small aortic valve annulus. (2) A small aortic annulus/descending aorta ratio is probably present at birth, and may decrease with increasing age. (3) In some patients with FSAS the aortic valve annulus is too small for simple resection of the fibroelastic tissue. A Konno operation is needed for these patients. (4) M-mode echocardiography has not been useful in identifying abnormally small aortic valve annulus in FSAS patients.

  16. Fluid dynamics of aortic root dilation in Marfan syndrome

    CERN Document Server

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

    2014-01-01

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

  17. Evaluation of fetal pulmonary hypoplasia by MR signal intensity

    International Nuclear Information System (INIS)

    Magnetic resonance (MR) imaging plays an important role in prenatal diagnosis and perinatal treatment. We correlated low intensity of the fetal lung after 25 weeks' gestation and neonatal respiratory distress in 35 fetuses. We employed MR imaging using the half-Fourier acquisition, single-shot, turbo spin-echo (HASTE) sequence. In all fetuses, signal intensity was lower in the lung than in the liver, and all infants subsequently had respiratory distress at birth. In 9 autopsy cases, pulmonary hypoplasia was established as the cause of death. Despite the small number of cases examined in this study, low signal intensity in the fetal lung may be a predictor of respiratory distress requiring mechanical ventilation at birth. (author)

  18. Quantification of abdominal aortic deformation after EVAR

    Science.gov (United States)

    Demirci, Stefanie; Manstad-Hulaas, Frode; Navab, Nassir

    2009-02-01

    Quantification of abdominal aortic deformation is an important requirement for the evaluation of endovascular stenting procedures and the further refinement of stent graft design. During endovascular aortic repair (EVAR) treatment, the aortic shape is subject to severe deformation that is imposed by medical instruments such as guide wires, catheters, and, the stent graft. This deformation can affect the flow characteristics and morphology of the aorta which have been shown to be elicitors for stent graft failures and be reason for reappearance of aneurysms. We present a method for quantifying the deformation of an aneurysmatic aorta imposed by an inserted stent graft device. The outline of the procedure includes initial rigid alignment of the two abdominal scans, segmentation of abdominal vessel trees, and automatic reduction of their centerline structures to one specified region of interest around the aorta. This is accomplished by preprocessing and remodeling of the pre- and postoperative aortic shapes before performing a non-rigid registration. We further narrow the resulting displacement fields to only include local non-rigid deformation and therefore, eliminate all remaining global rigid transformations. Finally, deformations for specified locations can be calculated from the resulting displacement fields. In order to evaluate our method, experiments for the extraction of aortic deformation fields are conducted on 15 patient datasets from endovascular aortic repair (EVAR) treatment. A visual assessment of the registration results and evaluation of the usage of deformation quantification were performed by two vascular surgeons and one interventional radiologist who are all experts in EVAR procedures.

  19. Repair of recurrent pseudoaneurysm of the mitral-aortic intervalvular fibrosa: Role of transesophageal echocardiography

    OpenAIRE

    Shreedhar S Joshi; Ashwini Thimmarayappa; P S Nagaraja; Jagadeesh, A. M.; Arul Furtado; Seetharam Bhat

    2014-01-01

    Pseudoaneurysm of mitral-aortic intervalvular fibrosa (P-MAIVF) is a rare cardiac surgical condition. P-MAIVF commonly occurs as a complication of aortic and mitral valve replacement surgeries. The surgical trauma during replacement of the valves weakens the avascular mitral and aortic intervalvular area. We present a case of P-MAIVF recurrence 5 years after a primary repair. Congestive cardiac failure was the presenting feature with mitral and aortic regurgitation. In view of the recurrence,...

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

    Institute of Scientific and Technical Information of China (English)

    Julia Massaad; Kelly Crawford

    2008-01-01

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

  1. [Nasal respiratory stenosis and maxillary hypoplasia. Changes after orthodontic treatment with rapid palatal expansion].

    Science.gov (United States)

    Piccini, A; Giorgetti, R; Fiorelli, G

    1989-01-01

    The relationship between hypoplasia of the upper maxillary bone and nasal respiratory insufficiency in a group of twenty infants with malocclusion being treated by rapid maxillary expansion (RME) were studied. Prior to treatment all patients presented endognatia with discrepancies of from -4 to -7 mm in the transverse basal skeleton. These were often associated with adenoid hypertrophy (70% of the cases), increased total nasal resistance (70%), oral respiration (80%) and middle ear diseases (30%). RME led to resolution of occlusion alterations in all cases and often also brought about a regression in adenoid hypertrophy (57% of the cases), normalization of the total nasal resistance (70%) and respiration (80%). These effects were achieved alone without association with any other form of medical or surgical E.N.T. treatment. The functional results confirmed by the radiological and clinical findings indicate an increase in the diameters of the nasal fossa and in the distance between the canines, between premolars and between molars as well as reduction in adenoid vegetation and in the diffuse hypertrophic tissues lining the naso-pharyngeal space. Nonetheless, hypoplasia of the upper maxillary bone and nasal respiratory insufficiency remain strictly linked and are bound to a variable, and at times uncertain, cause-effect relationship. Is nasal stenosis the moving force of maxillary-mandibular dysmorphism and gnatological dysfunction or does it result from an overall genetic conditioning of facial skeleton development? During their vast experience in adenoid and metadenoid pathologies in infancy the authors have, at times, observed significant maxillo-facial dysmorphisms. They have likewise found that "facies adenoidea" were not always associated with hypertrophy of the pharyngeal tonsil.(ABSTRACT TRUNCATED AT 250 WORDS)

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

    Science.gov (United States)

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

    2014-01-01

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

  3. A case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft

    Directory of Open Access Journals (Sweden)

    Miyake T

    2016-07-01

    Full Text Available Tomoko Miyake,1 Shota Kojima,1 Tetsuya Sugiyama,2 Mari Ueki,1 Jun Sugasawa,1 Hidehiro Oku,1 Kensuke Tajiri,1 Yuka Shigemura,3 Koichi Ueda,3 Atsuko Harada,4 Mami Yamasaki,4 Takumi Yamanaka,4 Hidetsuna Utsunomiya,5 Tsunehiko Ikeda1 1Department of Ophthalmology, Osaka Medical College, Takatsuki City, 2Nakano Eye Clinic of Kyoto Medical Co-operative, Kyoto, 3Department of Plastic and Reconstructive Surgery, Osaka Medical College, Takatsuki City, 4Department of Pediatric Neurosurgery, Takatsuki General Hospital, Takatsuki City, 5Department of Radiological Science, International University of Health and Welfare, Graduate School, Fukuoka, Japan Introduction: The incidence of facial cleft is rare and ranges between 1.43 and 4.85 per 100,000 births. To date, there have been few reports of detailed ophthalmologic examinations performed in cases of facial cleft. Here, we report a case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft. Case report: A 9-day-old female infant was delivered by cesarian section at 34 weeks of gestational age (the second baby of twins and weighed 2,276 g upon presentation. She had a facial cleft and ectrodactyly at birth. Right eye-dominant blepharophimosis was obvious. Examination of the right eye revealed inferior corneal opacity with vascularization, downward corectopia, and optic-nerve hypoplasia. The corneal diameter was 8 mm in both eyes, and tonometry by use of a Tono-Pen® XL (Reichert Technologies, Depew, NY, USA handheld applanation tonometer revealed that her intraocular pressure was 11–22 mmHg (Oculus Dexter and 8 mmHg (Oculus Sinister. B-mode echo revealed no differences in axial length between her right and left eyes. When she was 15–16 months old, we attempted to examine her eyes before she underwent plastic surgery under general anesthesia. She had a small optic disc in both eyes and the right-eye disc was tilted. After undergoing canthotomy, gonioscopy and ultrasound

  4. Radiological features of Goltz syndrome: Focal dermal hypoplasia. A report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Boothyrod, A.E.; Hall, C.M.

    1988-10-01

    Two female infants with Goltz syndrome (focal dermal hypoplasia) were recently investigated for severe feeding problems and failure to thrive. Both demonstrated severe skeletal malformations and marked gastrooesophageal reflux with laxity of the hiatus. One child (case 1) exhibited nasal regurgitation during feeding. Interestingly, both children had undergone surgery; Case 1 or a right parasagittal abdominal hernia associated with focal dermal hypoplasia of the abdominal wall and Case 2 for an exomphalos also associated with dermal hypoplasia. This observation suggests more widespread mesodermal abnormality. (orig./GDG).

  5. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... as atherosclerosis, high blood pressure, smokers, or a family history of abdominal aortic aneurysms. Today’s patient is ... be screened. In fact, patients who have a family history of aneurysm, men who are smoking over ...

  6. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... wall will actually thin out. And the big risk here is that if this gets too big ... to the aging baby boomers. Next slide. The risk factors for abdominal aortic aneurysms are males over ...

  7. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... atherosclerosis, high blood pressure, smokers, or a family history of abdominal aortic aneurysms. Today’s patient is a ... screened. In fact, patients who have a family history of aneurysm, men who are smoking over the ...

  8. Banding for type IA endoleak after endovascular abdominal aortic repair: An underexposed treatment option.

    Science.gov (United States)

    van Lammeren, G W; Ünlü, Ç; De Vries, J P P M

    2016-04-01

    More challenging abdominal aortic aneurysms with unfavorable proximal aortic neck anatomy are treated with endovascular means. As a consequence, proximal inadequate sealing may result in type IA endoleak, which in turn can lead to abdominal aortic aneurysm progression or rupture. The presence of type IA endoleak is an indication for secondary interventions. External aortic banding can be a good option to solve a type IA endoleak, but is underreported in literature; we present two cases and review literature.

  9. Incidental necropsy finding of a quadricuspid aortic valve

    NARCIS (Netherlands)

    van Rijswijk, Jan Willem; Willemink, Martin; Kluin, Jolanda; Vink, Aryan

    2015-01-01

    Quadricuspid aortic valve is a rare congenital cardiac malformation often associated with abnormal valve function. In this article, we present a case of quadricuspid aortic valve only diagnosed at the time of post-mortem examination. (C) 2015 Elsevier Inc. All rights reserved.

  10. Hypoplasia of the odontoid with atlanto-axial subluxation in Hurler's syndrome

    International Nuclear Information System (INIS)

    There appears to be an increased incidence of hypoplasia of the odontoid in Hurler's syndrome. As this predisposes to atlanto-axial subluxation, it should be sought in this mucopolysaccharidosis, as well as in Morquio's syndrome. (orig.)

  11. Prenatal prediction of pulmonary hypoplasia: clinical, biometric, and Doppler velocity correlates

    NARCIS (Netherlands)

    J.A.M. Laudij (Jacqueline); D. Tibboel (Dick); S.G.F. Robben (Simon); R.R. de Krijger (Ronald); M.A.J. de Ridder (Maria); J.W. Wladimiroff (Juriy)

    2002-01-01

    textabstractOBJECTIVES: To determine the value of pulmonary artery Doppler velocimetry relative to fetal biometric indices and clinical correlates in the prenatal prediction of lethal lung hypoplasia (LH) in prolonged (>1 week) oligohydramnios. METHODS: Forty-two singleton pregnanc

  12. Genetics Home Reference: intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and ...

    Science.gov (United States)

    ... abnormal side-to-side curvature of the spine ( scoliosis ) or thinning of the bones ( osteoporosis ). Adrenal hypoplasia ... health conditions: Diagnostic Tests Drug Therapy Surgery and Rehabilitation Genetic Counseling Palliative Care Related Information How are ...

  13. Acute Aortic Dissection Extending Into the Lung.

    Science.gov (United States)

    Makdisi, George; Said, Sameh M; Schaff, Hartzell V

    2015-07-01

    The radiologic manifestations of ruptured acute aortic dissection, Stanford type A aortic dissection, DeBakey type 1 can present in different radiographic scenarios with devastating outcomes. Here, we present a rare case of a 70-year-old man who presented to the emergency department with chest pain radiating to the back. A chest computed tomography scan showed a Stanford type A, DeBakey type 1, acute aortic dissection ruptured into the aortopulmonary window and stenosing the pulmonary trunk, both main pulmonary arteries, and dissecting the bronchovascular sheaths and flow into the pulmonary interstitium, causing pulmonary interstitial hemorrhage. The patient underwent emergent ascending aorta replacement with hemiarch replacement with circulatory arrest. The postoperative course was unremarkable. PMID:26140779

  14. Turner's tooth with unique radiographic presentation: a case report.

    Science.gov (United States)

    Lakshman, Anusha Rangare; Kanneppady, Sham Kishor; Castelino, Renita Lorina

    2014-01-01

    Hypoplasia--the result of a disruption in the enamel matrix formation process--causes a defect in the quality and thickness of enamel. Enamel formation is a complex and highly regulated process. Enamel defects have been associated with a broad spectrum of etiologies, including genetic, epigenetic, systemic, local, and environmental factors. An enamel defect in the permanent teeth caused by periapical inflammatory disease in the overlying primary tooth is referred to as Turner's tooth (also known as Turner's hypoplasia). This article presents a case of Turner's hypoplasia of the first mandibular premolar, with an unusual radiographic presentation. PMID:25184717

  15. Down-Regulation of Sonic Hedgehog Expression in Pulmonary Hypoplasia Is Associated with Congenital Diaphragmatic Hernia

    OpenAIRE

    Unger, Sharon; Copland, Ian; Tibboel, Dick; Post, Martin

    2003-01-01

    The pathogenesis of pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) is unknown. The sonic hedgehog (Shh) cascade is crucial for the patterning of the early respiratory system in mice. To establish whether Shh plays a role in the pathogenesis of lung hypoplasia in CDH, we investigated the gestation-specific expression of Shh in normal rat and human lungs using in situ hybridization and immunohistochemistry. The expression pattern was compared with that of age-matched...

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  17. Low-intensity fetal lungs on MRI may suggest the diagnosis of pulmonary hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kuwashima, S.; Nishimura, G.; Iimura, Fumitoshi; Kohno, Tatsuo; Kohno, Atsushi; Fujioka, Mutsuhisa [Department of Radiology, Dokkyo University School of Medicine, Tochigi-Ken (Japan); Watanabe, Hiroshi [Department of Obstetrics and Gynaecology, Dokkyo University School of Medicine, Tochigi-Ken (Japan)

    2001-09-01

    Background: Pulmonary hypoplasia is a common cause of neonatal death. Despite the recent advances in prenatal diagnosis with US, the diagnosis of pulmonary hypoplasia is difficult. The recent application of fast MR imaging may provide additional valuable information. Objective: To evaluate pulmonary hypoplasia in the fetus with MRI. Materials and methods: The subjects comprised 23 fetuses (18-40 weeks' gestation), including major anomalies diagnosed on fetal ultrasonography (n = 20), maternal abnormality (n = 2) and one normal twin. MRI was performed with a 1.5-T magnet and half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences. MR images were interpreted by three radiologists with special attention to the intensity of the lungs. The lung-to-liver intensity ratio was calculated by means of region-of-interest (ROI) analysis. The diagnosis of pulmonary hypoplasia depended on clinical, surgical and autopsy findings. Results: All fetuses with normal pulmonary development showed high intensity in the lung except for one fetus at 24 weeks' gestational age. All fetuses with pulmonary hypoplasia showed lung of low intensity. Conclusions: Low-intensity fetal lung on MRI imaging indicates pulmonary hypoplasia after 26 weeks' gestation. (orig.)

  18. Reoperative transapical transcatheter aortic valve replacement for central aortic regurgitation.

    Science.gov (United States)

    Zhu, Yuanjia; Kapadia, Samir; Krishnaswamy, Amar; Svensson, Lars G; Mick, Stephanie

    2016-09-01

    Paravalvular leak-related aortic regurgitation after transcatheter aortic valve replacement (TAVR) is a common complication and is associated with increased short- and long-term mortality. However, the impact of isolated central aortic regurgitation is unknown. We report a case of transapical (TA) TAVR with postprocedural central aortic regurgitation, who returned after two years with progression of regurgitation. A reoperative valve-in-valve TA-TAVR was performed. PMID:27405799

  19. Aortic regurgitation after transcatheter aortic valve replacement.

    Science.gov (United States)

    Werner, Nikos; Sinning, Jan-Malte

    2014-01-01

    Paravalvular aortic regurgitation (AR) negatively affects prognosis following transcatheter aortic valve replacement (TAVR). As transcatheter heart valves (THV) are anchored using a certain degree of oversizing at the level of the aortic annulus, incomplete stent frame expansion because of heavily annular calcifications, suboptimal placement of the prosthesis, and/or annulus-prosthesis size-mismatch can contribute to paravalvular AR with subsequent increased mortality risk. Echocardiography is essential to differentiate between transvalvular and paravalvular AR and to further elucidate the etiology of AR during the procedure. However, because echocardiographic quantification of AR in TAVR patients remains challenging, especially in the implantation situation, a multimodal approach to the evaluation of AR with use of hemodynamic measurements and imaging modalities is useful to precisely quantify the severity of AR immediately after valve deployment. "Next-generation" THVs are already on the market and first results show that paravalvular AR related to design modifications (eg, paravalvular space-fillers, full repositionability) are rarely seen in these valve types.  PMID:24632758

  20. The future of aortic surgery in Europe

    DEFF Research Database (Denmark)

    Czerny, Martin; Bachet, Jean; Bavaria, Joseph;

    2012-01-01

    At least every ten years, each specialty should reflect upon its past, its present and its future, in order to be able to reconfirm the direction in which it is headed, to adopt suggestions from inside and outside and, consequently, to improve. As such, the aim of this manuscript is to provide th...... in Europe, namely the Vascular Domain of the European Association of Cardio-Thoracic Surgery (EACTS)....... the interested reader with an overview of how aortic surgery and (perhaps more accurately) aortic medicine has evolved in Europe, and its present standing; also to provide a glimpse into the future, trying to disseminate the thoughts of a group of people actively involved in the development of aortic medicine...

  1. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... conditions or other significant medical problems, the American College of Cardiology recommends aortic valve replacement for basically ... more likely we see aortic stenosis. Again, patient education is part of the evaluation and management of ...

  2. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... North Carolina. My name is John Streitman and I'm a cardiothoracic surgeon here at the Heart ... the corrective surgery of aortic valve replacement. If I have aortic stenosis, are there any activities that ...

  3. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... of that slide, that demonstrates that patients with New York Heart Association class heart failure 1 and ... right down the aortic valve and that's the new aortic valve that Dr. Streitman's placed. And you ...

  4. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... to help prevent aortic stenosis? There's no prophylactic methods that anybody can take to prevent aortic stenosis ... it will be archived on the ORlive Web site and ORlive.com and, of course, a link ...

  5. Abdominal aortic feminism.

    Science.gov (United States)

    Mortimer, Alice Emily

    2014-01-01

    A 79-year-old woman presented to a private medical practice 2 years previously for an elective ultrasound screening scan. This imaging provided the evidence for a diagnosis of an abdominal aortic aneurysm (AAA) to be made. Despite having a number of recognised risk factors for an AAA, her general practitioner at the time did not follow the guidance set out by the private medical professional, that is, to refer the patient to a vascular specialist to be entered into a surveillance programme and surgically evaluated. The patient became symptomatic with her AAA, was admitted to hospital and found to have a tender, symptomatic, 6 cm leaking AAA. She consented for an emergency open AAA repair within a few hours of being admitted to hospital, despite the 50% perioperative mortality risk. The patient spent 4 days in intensive care where she recovered well. She was discharged after a 12 day hospital stay but unfortunately passed away shortly after her discharge from a previously undiagnosed gastric cancer.

  6. Aneurysms: thoracic aortic aneurysms.

    Science.gov (United States)

    Chun, Kevin C; Lee, Eugene S

    2015-04-01

    Thoracic aortic aneurysms (TAAs) have many possible etiologies, including congenital heart defects (eg, bicuspid aortic valves, coarctation of the aorta), inherited connective tissue disorders (eg, Marfan, Ehlers-Danlos, Loeys-Dietz syndromes), and degenerative conditions (eg, medial necrosis, atherosclerosis of the aortic wall). Symptoms of rupture include a severe tearing pain in the chest, back, or neck, sometimes associated with cardiovascular collapse. Before rupture, TAAs may exert pressure on other thoracic structures, leading to a variety of symptoms. However, most TAAs are asymptomatic and are found incidentally during imaging for other conditions. Diagnosis is confirmed with computed tomography scan or echocardiography. Asymptomatic TAAs should be monitored with imaging at specified intervals and patients referred for repair if the TAAs are enlarging rapidly (greater than 0.5 cm in diameter over 6 months for heritable etiologies; greater than 0.5 cm over 1 year for degenerative etiologies) or reach a critical aortic diameter threshold for elective surgery (5.5 cm for TAAs due to degenerative etiologies, 5.0 cm when associated with inherited syndromes). Open surgery is used most often to treat asymptomatic TAAs in the ascending aorta and aortic arch. Asymptomatic TAAs in the descending aorta often are treated medically with aggressive blood pressure control, though recent data suggest that endovascular procedures may result in better long-term survival rates. PMID:25860136

  7. Adventitial vasa vasorum arteriosclerosis in abdominal aortic aneurysm.

    Directory of Open Access Journals (Sweden)

    Hiroki Tanaka

    Full Text Available Abdominal aortic aneurysm (AAA is a common disease among elderly individuals. However, the precise pathophysiology of AAA remains unknown. In AAA, an intraluminal thrombus prevents luminal perfusion of oxygen, allowing only the adventitial vaso vasorum (VV to deliver oxygen and nutrients to the aortic wall. In this study, we examined changes in the adventitial VV wall in AAA to clarify the histopathological mechanisms underlying AAA. We found marked intimal hyperplasia of the adventitial VV in the AAA sac; further, immunohistological studies revealed proliferation of smooth muscle cells, which caused luminal stenosis of the VV. We also found decreased HemeB signals in the aortic wall of the sac as compared with those in the aortic wall of the neck region in AAA. The stenosis of adventitial VV in the AAA sac and the malperfusion of the aortic wall observed in the present study are new aspects of AAA pathology that are expected to enhance our understanding of this disease.

  8. 3D image analysis of abdominal aortic aneurysm

    Science.gov (United States)

    Subasic, Marko; Loncaric, Sven; Sorantin, Erich

    2002-05-01

    This paper presents a method for 3-D segmentation of abdominal aortic aneurysm from computed tomography angiography images. The proposed method is automatic and requires minimal user assistance. Segmentation is performed in two steps. First inner and then outer aortic border is segmented. Those two steps are different due to different image conditions on two aortic borders. Outputs of these two segmentations give a complete 3-D model of abdominal aorta. Such a 3-D model is used in measurements of aneurysm area. The deformable model is implemented using the level-set algorithm due to its ability to describe complex shapes in natural manner which frequently occur in pathology. In segmentation of outer aortic boundary we introduced some knowledge based preprocessing to enhance and reconstruct low contrast aortic boundary. The method has been implemented in IDL and C languages. Experiments have been performed using real patient CTA images and have shown good results.

  9. Idiopathic thoracic aortic aneurysm at pediatric age.

    Science.gov (United States)

    Marín-Manzano, E; González-de-Olano, D; Haurie-Girelli, J; Herráiz-Sarachaga, J I; Bermúdez-Cañete, R; Tamariz-Martel, A; Cuesta-Gimeno, C; Pérez-de-León, J

    2009-03-01

    A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.

  10. Infective endocarditis following transcatheter aortic valve replacement-

    DEFF Research Database (Denmark)

    Loh, Poay Huan; Bundgaard, Henning; S�ndergaard, Lars

    2013-01-01

    Transcatheter aortic valve replacement (TAVR) can improve the symptoms and prognosis of patients with severe aortic stenosis who, due to a high expected operative risk, would not have otherwise been treated surgically. If these patients develop prosthetic valve endocarditis, their presentations may...... be atypical causing a delay in the diagnosis and treatment. The management is also complicated by their comorbidities, and surgical treatment may not be feasible leading to a significant morbidity and mortality. We describe a case of an 85-year-old man with TAVI prosthetic valve endocarditis successfully...

  11. 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

    Aortic coarctation (AC) is a congenital aortic narrowing. We describe for the first time the findings obtained by unenhanced post mortem computed tomography (PMCT) in a case where the death was caused by cardiac tamponade from a ruptured aneurysmal dilatation of the ascending aorta and the aortic...... and the aortic arch without aortic dissection associated with AC is an uncommon cause of haemopericardium that has only been described a few times before. The case is discussed in relation to other reported cases and in the context of the present understanding of this condition....

  12. ED 02-1 ACUTE AORTIC SYNDROME.

    Science.gov (United States)

    Song, Jae-Kwan

    2016-09-01

    The successful clinical introduction of various non-invasive imaging modalities has contributed to the establishment of 'acute aortic syndrome', a relatively new clinical syndrome incorporating several disease entities with similar clinical features (at the time of clinical presentation). Aortic dissection (AD) with intimal flap and two aortic channels (true and false lumens) is the most important disease entity in acute aortic syndrome. Clinical characteristics of AD have been established, with standardized treatment strategies depending on the affected site of the aorta. For the past several decades, variant forms of classic AD, including aortic intramural hematoma (IMH) and incomplete dissection, have been increasingly diagnosed in routine clinical practice worldwide, and imaging findings characteristic of these variant forms have been established for their differential diagnosis. As imaging findings, rather than clinical features, are critical for the differential diagnosis of acute aortic syndrome, careful interpretation of imaging results is necessary for accurate diagnosis and better clinical decision-making, thus improving patient outcomes. IMH is probably the most important variant form of classic AD and has been the source of intense controversy regarding its pathogenesis and optimal treatment options. Clinical reports regarding outcomes of patients with IMH from different centers have shown conflicting results and risk stratification based on imaging findings remains a challenging clinical issue.Intimal tears can have a very wide spectrum. For example, a stellate or linear intimal tear can involve the exposure of the underlying aortic media or adventitial layers but without the progression and separation of the medial layers, resulting in extensive undermining of the intimal layers. This incomplete dissection, characterized by an intimal tear without an intimal flap or hematoma, was reported to be present in 9 of 181 (5%) consecutive patients with

  13. Adrenal hypoplasia congenita: a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism

    Directory of Open Access Journals (Sweden)

    Marta Loureiro

    2015-09-01

    Full Text Available Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG, confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

  14. [Albert Einstein and his abdominal aortic aneurysm].

    Science.gov (United States)

    Cervantes Castro, Jorge

    2011-01-01

    The interesting case of Albert Einstein's abdominal aortic aneurysm is presented. He was operated on at age 69 and, finding that the large aneurysm could not be removed, the surgeon elected to wrap it with cellophane to prevent its growth. However, seven years later the aneurysm ruptured and caused the death of the famous scientist.

  15. Diagnostic imaging of acute aortic dissection

    International Nuclear Information System (INIS)

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

  16. Dental enamel Hypoplasia. Investigations on the Bones Exhumed from the Medieval Necropole of Lozova (Republic of Moldova, XIVth–XVth Centuries

    Directory of Open Access Journals (Sweden)

    Robert Daniel Simalcsik

    2014-10-01

    Full Text Available Dental hypoplasia is a developmental anomaly based on perturbations of amelogenesis. Hypoplasia defects are part of the unspecific quantitative indicators for the state of health and / or nutritional state during the formation of the dental buds. It is a response of the human organism to physiological stress. The incidence of this dysplasia in a past population can indicate its biological frailty in its attempt to adapt to the environmental changes. The osteological material was excavated in the interval 2010 – 2011 by archaeologists from the Archaeology Centre in Chisinau, from the Medieval cemetery of Lozova (Straseni County, Republic of Moldova, dated for the XIVth and XVth centuries. Fifty one skeletons from 50 inhumation graves have been excavated and analyzed so far. Only 40 individuals had most of their teeth present. The enamel hypoplasia is of linear transversal type, located on the labial surface of the dental crowns, in the median third. The canine is the most affected tooth, followed by the incisors. The incidence of dental enamel hypoplasia at population level (based on the data collected and on the number of graves excavates so far, which does not illustrate the entire population of the cemetery is 7.5%. The incidence of dental caries is 23.53%, of cribra orbitalia – 11.75%, and of cribra cranii externa – 1.96%. The results obtained for a relatively small rural community illustrate a good adaptation to the stressing environmental factors. The possible malnutrition and illness episodes suffered during early childhood were recovered along the growth and development processes.

  17. Inflammatory aortic aneurysms

    DEFF Research Database (Denmark)

    Bitsch, M; Nørgaard, H H; Røder, O;

    1997-01-01

    operated on for abdominal aortic aneurysms without PF in the same period, served as reference group. RESULTS: Preoperative thickness of PF was assessed as > 1 cm in 11 and cm in 10 patients. Ureterolysis was performed in seven patients where the fibrosis caused ureteral obstruction. Postoperative CT...

  18. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... to repair an abdominal aortic aneurysm using a technology called an “endograft,” which is sometimes called the “ ... separate area, and it’s because of this small technology that allows you to do this. Exactly. So ...

  19. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... an abdominal aortic aneurysm. Normally this procedure takes us about 45 minutes to an hour of doctor work time, which is, I think, a fairly quick procedure to replace a major life-threatening problem in the patient’s abdomen. So you can see ...

  20. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... the arteries, which is known as atherosclerosis, high blood pressure, smokers, or a family history of abdominal aortic ... imaging. We can integrate ultrasound imaging, the patient’s blood pressure, and so it’s a little bit like being ...

  1. Hypoplasia of the sphenoid sinuses as a diagnostic tool in cystic fibrosis

    International Nuclear Information System (INIS)

    Purpose: To measure and compare the size of the sphenoid sinuses in patients with cystic fibrosis (CF) to patients with inflammatory sinonasal disease, and to correlate the size with number of CF mutations in each patient. Material and Methods: Ninety-six CF patients aged 5-47 years (median 19 years) and 130 control patients aged 7-51 years (median 32 years) were examined using coronal CT of the paranasal sinuses. In each patient, the CT image with the largest coronal area of the sphenoid sinuses was scanned into a Macintosh computer with image processing and analysis software. Largest coronal area and largest circumference of the right and left sphenoid sinuses were automatically measured. Additionally, antero-posterior extension of the sphenoid sinuses was calculated from the lateral scanograms. CF patients were grouped according to number of confirmed mutations (CF-0, CF-1, or CF-2). Results: CF patients generally had small sphenoid sinuses. The largest difference for all parameters were observed between the CF-2 and the control groups (p<0.0001). No CF-2 patient had pneumatization beyond the presphenoid. The CF-0 and CF-1 groups consisted of two populations, one overlapping the CF-2 group and another overlapping the control group. Conclusion: Hypoplasia of the sphenoid sinuses is a characteristic finding in CF patients. When pneumatization of the basisphenoid is present, the existing CF diagnosis should be questioned. (orig.)

  2. Enamel hypoplasias and physiological stress in the Sima de los Huesos Middle Pleistocene hominins.

    Science.gov (United States)

    Cunha, E; Rozzi, F Ramirez; Bermúdez de Castro, J M; Martinón-Torres, M; Wasterlain, S N; Sarmiento, S

    2004-11-01

    This study presents an analysis of linear enamel hypoplasias (LEH) and plane-form defects (PFD) in the hominine dental sample from the Sima de los Huesos (SH) Middle Pleistocene site in Atapuerca (Spain). The SH sample comprises 475 teeth, 467 permanent and 8 deciduous, belonging to a minimum of 28 individuals. The method for recording PFD and LEH is discussed, as well as the definition of LEH. The prevalence of LEH and PFD in SH permanent dentition (unilateral total count) is 4.6% (13/280). Only one deciduous tooth (lower dc) showed an enamel disruption. Prevalence by individual ranges from 18.7-30%. The most likely explanation for the relatively low LEH and PFD prevalence in the SH sample suggests that the SH population exhibited a low level of developmental stress. The age at occurrence of LEH and PFD was determined by counting the number of perikymata between each lesion and the cervix of the tooth. Assuming a periodicity of nine days for the incremental lines, the majority of LEH in the SH sample occurred during the third year of life and may be related to the metabolic stress associated with weaning. PMID:15386251

  3. Endovascular aortic repair: First twenty years

    Directory of Open Access Journals (Sweden)

    Končar Igor

    2012-01-01

    Full Text Available Endovascular aortic/aneurysm repair (EVAR was introduced into clinical practice at the beginning of the nineties. Its fast development had a great influence on clinicians, vascular surgeons and interventional radiologists, educational curriculums, patients, industry and medical insurance. The aim of this paper is to present the contribution of clinicians and industry to the development and advancement of endovascular aortic repair over the last 20 years. This review article presents the development of EVAR by focusing on the contribution of physicians, surgeons and interventional radiologists in the creation of the new field of vascular surgery termed hybrid vascular surgery, and also the contribution of technological advancement by a significant help of industrial representatives – engineers and their counselors. This article also analyzes studies conducted in order to compare the successfulness of EVAR with up-to-now applied open surgical repair of aortic aneurysms, and some treatment techniques of other aortic diseases. During the first two decades of its development the EVAR method was rapidly progressing and was adopted concurrently with the expansion of technology. Owing to large randomized studies, early and long-term results indicate specific complications of this method, thus influencing further technological improvement and defining risk patients groups in whom the use of the technique should be avoided. Good results are insured only in centers, specialized in vascular surgery, which have on their disposal adequate conditions for solving all complications associated with this method.

  4. Thrombocytosis following splenectomy and aortic valve replacement for idiopathic thrombocytopaenic purpura with bicuspid aortic valve

    Directory of Open Access Journals (Sweden)

    Sarika Katiyar

    2015-01-01

    Full Text Available Idiopathic thrombocytopaenic purpura (ITP patients are at high risk for complications during and after cardiac surgeries involving cardiopulmonary bypass. The main clinical problem of primary ITP is an increased risk of bleeding although bleeding may not always be present. More recently, thrombosis has become appreciated as another potential complication of the procedure. We report a 22-year-old female patient with ITP with bicuspid aortic valve and splenomegaly, who underwent uncomplicated aortic valve replacement and splenectomy simultaneously. She was readmitted with chest pain due to coronary thrombosis following splenectomy which made the management difficult. We describe our experience in managing this patient who presented with thrombotic complication rather than bleeding in post-operative period and the challenges met in maintaining appropriate anticoagulation for aortic valve replacement as well as thrombosis, post-splenectomy

  5. First direct aortic retrievable transcatheter aortic valve implantation in humans.

    Science.gov (United States)

    Chandrasekhar, Jaya; Glover, Chris; Labinaz, Marino; Ruel, Marc

    2014-11-01

    We describe 2 cases in which transcatheter aortic valve implantation was performed with a Portico prosthesis (St Jude Medical, St Paul, MN) through a direct aortic approach. In 1 of the cases, prosthesis retrieval was needed during the procedure and was essential to the successful outcome. This is the first report, to our knowledge, of direct aortic Portico prosthesis implantation, and it highlights the significance of the retrievable nature of this device. PMID:25442452

  6. Histopathological study of congenital aortic valve malformations in 32 children

    Institute of Scientific and Technical Information of China (English)

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

    2007-01-01

    The histopathological characteristics of congenital aortic valve malformations in children were investigated.All the native surgically excised aortic valves from 32 pediatric patients suffering from symptomatic aortic valve dysfunction due to congenital aortic valve malformations between January 2003 and December 2005 were studied macroscopically and microscopically.The patients' medical records were reviewed and the clinical information was extracted.The diagnosis was made by the clinical presentation,preoperative echocardiography,intraoperative examination,and postoperative histopathological study,excluding rheumatic ot degenerative aortic valve diseases,infective endocarditis and primary connective tissue disorders,e.g.Marfan syndrome.Among 32 children with congenital aortic valve malformations,the age was ranged from six to 18 years,with a mean of 14.9 years,and there were 27 boys and five girls (male:female = 5.4:1).There were five cases of aortic stenosis (AS,15.62%),25 cases of aortic insufficiency (AI,78.13 %)and two cases of AS-AI (6.25%),without other valve diseases.Twenty cases still had other congenital heart diseases:ventricular septal defect (19 cases),patent ductus arteriosus (two cases),double-chambered right ventricle (one case),aneurysm of the right anterior aortic sinus of valsalva (three cases).Histopathological examination indicated that the cusps became thickening with unequal size,irregular shape (coiling and prolapse edge),enhanced hardness,and partly calcification.Microscopic investigation revealed the unsharp structure of valve tissue,fibrosis,myxomatous,reduced collagen fiber,rupture of elastic fibers,different degrees of infiltration of inflammatory cells,secondary calcareous and lipid deposit,and secondary fibrosis.Congenital aortic valve malformations in children involve males more than females,mostly associated with other congenital heart diseases.Aortic insufficiency is more common in children with congenital aortic valve

  7. Enlightenment from a small but rapidly evolving penetrating aortic ulcer.

    Science.gov (United States)

    Tan, Guangyi; Tang, Wenyi; Chen, Jian

    2016-07-01

    Penetrating aortic ulcer (PAU) is a pathologic type of acute aortic syndrome and usually locates in the descending aorta. The presentation, behavior and natural history of this disease process have not been clear. Here we report a case in which a rapidly evolving PAU in descending aorta needed aggressive percutaneous interventional treatment. The present case with its unique scenario might draw clinicians' attention on a "beyond the guidelines" issue. PMID:26961076

  8. Autologous adventitial overlay method reinforces anastomoses in aortic surgery.

    Science.gov (United States)

    Minato, Naoki; Okada, Takayuki; Sumida, Tomohiko; Watanabe, Kenichi; Maruyama, Takahiro; Kusunose, Takashi

    2014-05-01

    In this study, we present an inexpensive and effective method for providing a secure and hemostatic anastomosis using autologous adventitia obtained from a dissected or aneurysmal wall. The resected aortic wall is separated between the adventitia and media, and a soft, 2 × 10-cm adventitial strip is overlaid to cover the anastomotic margin. A graft is sutured to the aortic stump. This autologous adventitial overlay method can inexpensively and strongly reinforce the anastomosis during aortic surgery for dissection or aneurysm and will contribute to anastomotic hemostasis and long-term stability.

  9. Mitral-aortic annular enlargement: modification of Manouguian's technique

    Directory of Open Access Journals (Sweden)

    Costa Mario Gesteira

    2002-01-01

    Full Text Available We hereby present a technical modification for mitral-aortic annular enlargement. The mitral valve is replaced through the retro-septal approach, avoiding patches for left atrial roof closure. We report a mitral-aortic valve replacement in a patient whose original annuli would preclude adequate prostheses. The simultaneous annular enlargement may be necessary for avoiding patient-prosthesis mismatch and for reconstructing destroyed mitral and aortic annuli. The technique may minimize the risk of bleeding and of paravalvular leakage, using an approach well known to cardiac surgeons.

  10. Bone marrow hypoplasia and intestinal crypt cell necrosis associated with fenbendazole administration in five painted storks.

    Science.gov (United States)

    Weber, Martha A; Terrell, Scott P; Neiffer, Donald L; Miller, Michele A; Mangold, Barbara J

    2002-08-01

    Five painted storks were treated with fenbendazole for 5 days for internal parasitism. Four birds died following treatment. Profound heteropenia was a consistent finding in all samples evaluated; additionally, the 1 surviving bird had progressive anemia. Consistent necropsy findings in the 4 birds that died were small intestinal crypt cell necrosis and severe bone marrow depletion and necrosis. Fenbendazole has been associated with bone marrow hypoplasia and enteric damage in mammals and other species of birds. The dosages of fenbendazole used in birds are often substantially higher than those recommended for mammals, which may contribute to bone marrow hypoplasia and intestinal crypt cell necrosis associated with fenbendazole administration in birds.

  11. Turner's hypoplasia and non-vitality: A case report of sequelae in permanent tooth

    OpenAIRE

    2010-01-01

    Hypoplasia is the result of disruption in the process of enamel matrix formation, which in turn causes defect in quality and thickness of enamel. Four cases of Turner′s hypoplastic teeth with a previous history of trauma/infection in their primary predecessors at the age of 2-3 years have been reported. These hypoplastic teeth had turned non-vital without any carious insult, cavitation or further trauma. This article thereby stresses the importance of early detection of enamel hypoplasia and ...

  12. Effect of abdominal aortic grafts on aortic stiffness and central hemodynamics.

    Science.gov (United States)

    Lantelme, Pierre; Dzudie, Anastase; Milon, Hugues; Bricca, Giampiero; Legedz, Liliana; Chevalier, Jean-Michel; Feugier, Patrick

    2009-06-01

    Graft-prosthesis and stentgraft placements are effective modalities for treating abdominal aortic aneurysm, but related changes in arterial stiffness are not well established. The present study sought to assess aortic stiffness after aneurism repair by measuring pulse wave velocity (PWV). The graft-related variation of carotid-femoral PWV was compared with that of carotid-radial PWV, the latter being unaffected by vascular treatment. The secondary objective was to evaluate potential differences between graft-prosthesis and stentgraft in terms of aortic stiffness and augmentation index, a composite indicator integrating wave reflexion. Fifty patients were included (39 had a graft-prosthesis and 11 had a stentgraft). In the whole group and after a median postoperative follow-up of 47 days, carotid-femoral PWV increased by +1.0 m/s [-12.3, +10.3], while carotid-radial PWV slightly decreased by -0.3 m/s [-4.4; +3.5] (P = 0.001). The effect of the type of prosthesis on the PWV was not significant. Nevertheless, the augmentation index increased after stentgraft implantation (+4% [-10; +17]) and decreased after graft-prosthesis placement (-8.5% [-47; +17]) (P aortic grafts on aortic stiffness. Besides, it suggests that stentgraft increases reflected waves more than graft-prostheses. These changes of vascular properties may influence the outcomes after surgery. PMID:19342960

  13. Tobacco smoking and aortic aneurysm

    DEFF Research Database (Denmark)

    Sode, Birgitte F; Nordestgaard, Børge G; Grønbæk, Morten;

    2012-01-01

    BACKGROUND: We determined the predictive power of tobacco smoking on aortic aneurysm as opposed to other risk factors in the general population. METHODS: We recorded tobacco smoking and other risk factors at baseline, and assessed hospitalization and death from aortic aneurysm in 15,072 individuals...... General Population Study, respectively. According to the magnitude of the hazard ratios, tobacco consumption was the most important risk factor for hospitalization and death from aortic aneurysm, followed by male sex and hypertension in both cohorts. The population attributable risk of aortic aneurysm...... outcomes due to tobacco consumption was 64% and 47% in the Copenhagen City Heart Study and Copenhagen General Population Study, respectively, and ranked highest among population attributable risks of aortic aneurysm in both cohorts. The absolute 10-year risk for hospitalization or death from aortic...

  14. Aortic valve replacement

    DEFF Research Database (Denmark)

    Kapetanakis, Emmanouil I; Athanasiou, Thanos; Mestres, Carlos A;

    2008-01-01

    BACKGROUND AND AIMS OF THE STUDY: Prompted by anecdotal evidence and observations by surgeons, an investigation was undertaken into the potential differences in implanted aortic valve prosthesis sizes, during aortic valve replacement (AVR) procedures, between northern and southern European...... countries. METHODS: A multi-institutional, non-randomized, retrospective analysis was conducted among 2,932 patients who underwent AVR surgery at seven tertiary cardiac surgery centers throughout Europe. Demographic and perioperative variables including valve size and type, body surface area (BSA) and early......: Prostheses with diameter valve size was also smaller in southern compared to northern European patients (21.6 +/- 2.1 mm versus 23.4 +/- 2.2 mm, p

  15. Micromanaging Abdominal Aortic Aneurysms

    Directory of Open Access Journals (Sweden)

    Lars Maegdefessel

    2013-07-01

    Full Text Available The contribution of abdominal aortic aneurysm (AAA disease to human morbidity and mortality has increased in the aging, industrialized world. In response, extraordinary efforts have been launched to determine the molecular and pathophysiological characteristics of the diseased aorta. This work aims to develop novel diagnostic and therapeutic strategies to limit AAA expansion and, ultimately, rupture. Contributions from multiple research groups have uncovered a complex transcriptional and post-transcriptional regulatory milieu, which is believed to be essential for maintaining aortic vascular homeostasis. Recently, novel small noncoding RNAs, called microRNAs, have been identified as important transcriptional and post-transcriptional inhibitors of gene expression. MicroRNAs are thought to “fine tune” the translational output of their target messenger RNAs (mRNAs by promoting mRNA degradation or inhibiting translation. With the discovery that microRNAs act as powerful regulators in the context of a wide variety of diseases, it is only logical that microRNAs be thoroughly explored as potential therapeutic entities. This current review summarizes interesting findings regarding the intriguing roles and benefits of microRNA expression modulation during AAA initiation and propagation. These studies utilize disease-relevant murine models, as well as human tissue from patients undergoing surgical aortic aneurysm repair. Furthermore, we critically examine future therapeutic strategies with regard to their clinical and translational feasibility.

  16. A Migrated Aortic Stent Graft Causing Erosive Spondylopathy

    Energy Technology Data Exchange (ETDEWEB)

    Gestrich, Christopher, E-mail: christopher.gestrich@ukb.uni-bonn.de; Probst, Chris, E-mail: chris.probst@ukb.uni-bonn.de [Universitaetsklinikum Bonn, Department of Cardiac Surgery (Germany); Wilhelm, Kai, E-mail: kai.wilhelm@ek-bonn.de [Johanniterkrankenhaus Bonn, Department of Radiology (Germany); Schiller, Wolfgang, E-mail: wolfgang.schiller@ukb.uni-bonn.de [Universitaetsklinikum Bonn, Department of Cardiac Surgery (Germany)

    2013-12-15

    We report about a patient presenting with back pain 4 months after an uneventful endovascular implantation of an aortic stent graft. Computed tomography scan revealed a migration of the stent with consecutive endoleakage, kink formation, and movement of the stent toward the spine, which caused destruction of the aortic wall as well as vertebral necrosis. Explantation of the stent and replacement of the native aorta relieved the patient of his symptoms.

  17. EXOSC8 mutations alter mRNA metabolism and cause hypomyelination with spinal muscular atrophy and cerebellar hypoplasia

    Science.gov (United States)

    Boczonadi, Veronika; Müller, Juliane S.; Pyle, Angela; Munkley, Jennifer; Dor, Talya; Quartararo, Jade; Ferrero, Ileana; Karcagi, Veronika; Giunta, Michele; Polvikoski, Tuomo; Birchall, Daniel; Princzinger, Agota; Cinnamon, Yuval; Lützkendorf, Susanne; Piko, Henriett; Reza, Mojgan; Florez, Laura; Santibanez-Koref, Mauro; Griffin, Helen; Schuelke, Markus; Elpeleg, Orly; Kalaydjieva, Luba; Lochmüller, Hanns; Elliott, David J.; Chinnery, Patrick F.; Edvardson, Shimon; Horvath, Rita

    2014-01-01

    The exosome is a multi-protein complex, required for the degradation of AU-rich element (ARE) containing messenger RNAs (mRNAs). EXOSC8 is an essential protein of the exosome core, as its depletion causes a severe growth defect in yeast. Here we show that homozygous missense mutations in EXOSC8 cause progressive and lethal neurological disease in 22 infants from three independent pedigrees. Affected individuals have cerebellar and corpus callosum hypoplasia, abnormal myelination of the central nervous system or spinal motor neuron disease. Experimental downregulation of EXOSC8 in human oligodendroglia cells and in zebrafish induce a specific increase in ARE mRNAs encoding myelin proteins, showing that the imbalanced supply of myelin proteins causes the disruption of myelin, and explaining the clinical presentation. These findings show the central role of the exosomal pathway in neurodegenerative disease. PMID:24989451

  18. Parvovirus associated cerebellar hypoplasia and hydrocephalus in day-old broiler chickens

    Science.gov (United States)

    Cerebellar hypoplasia and hydrocephalus were detected in day-old broiler chickens. Brains of chickens evaluated at necropsy appeared to be abnormal; some were disfigured and cerebellae appeared to be smaller than normal. Histopathologic examination of brains revealed cerebellar folia that were sho...

  19. CARTILAGE HAIR HYPOPLASIA, METAPHYSEAL CHONDRODYSPLASIA TYPE MCKUSICK - DESCRIPTION OF 7 PATIENTS AND REVIEW OF THE LITERATURE

    NARCIS (Netherlands)

    VANDERBURGT, [No Value; HARALDSSON, A; OOSTERWIJK, JC; VANESSEN, AJ; WEEMAES, C; HAMEL, B

    1991-01-01

    We describe 7 cases of cartilage hair hypoplasia (CHH) with emphasis on the clinical and immunological aspects. The literature on CHH is reviewed and symptoms in 63 non-Amish cases are summarized. In this autosomal recessive disorder the immunodeficiency, hair abnormalities, and severity of skeletal

  20. Concurrent occipital hypoplasia, occipital dysplasia, syringohydromyelia, and hydrocephalus in a Yorkshire terrier

    OpenAIRE

    Cagle, Laura

    2010-01-01

    Magnetic resonance imaging of a 7.5-year-old neutered male Yorkshire terrier with mild generalized ataxia and intermittent neck scratching led to a diagnosis of caudal occipital malformation and syringohydromyelia. Surgical exploration led to a diagnosis of occipital dysplasia with concurrent occipital hypoplasia. Following a dorsal laminectomy of the first cervical vertebra there was no progression or improvement a month later.

  1. Bilateral mandibular distraction in micrognathism or hypoplasia of mandible, hazrat fatemeh hospital

    Directory of Open Access Journals (Sweden)

    Mohammad-Esmaiil Hassani

    2014-01-01

    Conclusion: The Bilateral Distraction Oseogenesis method can bring forwards the retruded mandible effectively and its results has no difference with older methods. It is an effective and reliable procedure for hypoplasia of Mandible and the results are comparable with older methods.

  2. Growth hormone treatment in cartilage-hair hypoplasia: effects on growth and the immune system.

    NARCIS (Netherlands)

    Bocca, G.; Weemaes, C.M.R.; Burgt, C.J.A. van der; Otten, B.J.

    2004-01-01

    Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disorder characterized by metaphyseal chondrodysplasia with severe growth retardation and impaired immunity. We studied the effects of growth hormone treatment on growth parameters and the immune system in four children with CHH. The effe

  3. Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI

    Energy Technology Data Exchange (ETDEWEB)

    Katorza, Eldad; Nahama-Allouche, Catherine; Ducou le Pointe, Hubert; Garel, Catherine [Hopital d' Enfants Armand-Trousseau, Service de Radiologie, Paris (France); Castaigne, Vanina [Hopital Saint-Antoine, Service de Gynecologie-Obstetrique, Paris (France); Gonzales, Marie; Marlin, Sandrine [Hopital d' Enfants Armand-Trousseau, Service de Genetique et Embryologie medicales, Paris (France); Galliani, Eva [Hopital d' Enfants Armand-Trousseau, Service de Chirurgie maxillo-faciale, Paris (France); Jouannic, Jean-Marie; Rosenblatt, Jonathan [Hopital d' Enfants Armand-Trousseau, Service de Gynecologie-Obstetrique, Centre pluridisciplinaire de diagnostic prenatal, Paris (France)

    2011-05-15

    Analysis of the middle ear with fetal MRI has not been previously reported. To show the contribution of fetal MRI to middle ear imaging. The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks' gestation, respectively. In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US. The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia. (orig.)

  4. Detection of hypoplasia of bony cochlear nerve canal by virtual endoscopy: a pilot study

    Energy Technology Data Exchange (ETDEWEB)

    Quan Yong; Wu Lebin; Gong Wuxian; Gong Ruozhen (Shandong Medical Imaging Research Institute, Shandong Univeristy, Jinan, Shandong (China)), email: grzh99@yahoo.com.cn; Zu Zushan (Dept. of Radiology, Wendeng Central Hospital, Weifang Medical College, Weihai (China))

    2011-09-15

    Background: Dimensions of the bony cochlear nerve canal (BCNC) have been proposed as a potential diagnostic standard for hypoplasia of BCNC, but the standard remains inconsistent. We have previously found that a helix-like shape appears in normal BCNCs at VE images, whereas, the sign does not appear in some hypoplastic BCNCs. Purpose: To retrospectively examine the feasibility of computed tomographic (CT) virtual endoscopy (VE) in the evaluation of hypoplasia of BCNC on the basis of absence of a helix-like shape. Material and Methods: Twenty ears in 14 consecutive patients (mean age 5.5 years, range 1-15 years, 6 boys, 8 girls) diagnosed with hypoplasia of BCNC were included in this work. One hundred ears in 50 gender- and age-matched individuals (mean age 6.6 years, range 1-15 years, 29 boys, 21 girls) without inner ear disease and internal auditory canal (IAC) malformations served as controls. The presence or absence of a helix-like shape was evaluated by two independent reviewers. The value of VE for the diagnosis of hypoplasia of BCNC was assessed with clinical results and routine radiologic evaluation as the reference standard. Inter-observer agreement was calculated. Sensitivity, specificity, and accuracy were selected to test the diagnostic ability of the VE. Results: Absence of a helix-like shape was found in the cochlear area of 17 of 20 ears in patients with hypoplasia of BCNC but in none of the control subjects. Inter-observer agreement was substantial (? = 0.773). The diagnostic rates of absence of a helix-like shape for hypoplasia of BCNC in terms of sensitivity, specificity, and accuracy were 85%, 100%, and 98%, respectively. There were significant differences between the two groups with respect to VE findings for absence of a helix-like shape (P < 0.001). Conclusion: The absence of a helix-like shape at VE images may be used as a potentially useful sign in the diagnosis of hypoplasia of BCNC

  5. Chest radiograph usefulness in the diagnosis of acute aortic dissection

    Institute of Scientific and Technical Information of China (English)

    Nancy Welch; Chat Dang; Carlton Allen; Robert Cook

    2005-01-01

    Objective To assess the diagnostic value of chest radiographs in patients presenting to a busy inner-city Emergency Department with subsequently proven acute aortic dissection. Methods A retrospective review of initial chest radiographs and charts of patients with the confirmed diagnosis of acute aortic dissection was done for a period of 5 years from 1998 to 2003. A comparison was made between the initial readings of chest radiographs prior to confirmation of the aortic dissection, and a retrospective review of the same radiographs by two board-certified radiologists with special attention to the classic findings of acute aortic dissection identifiable on plain films. Results The charts of nine patients (four men, five women) with proven acute aortic dissection were reviewed. All nine patients were suspected of having acute aortic dissection based on presenting history and symptoms of chest pain (66% ), migratory pain (89% ), back pain (89% ), and the abruptness of onset of pain (89% ). Initial plain portable chest X-rays were obtained in the Emergency Department in all nine patients. Six of nine (67%) radiographs were read as normal, while three (33%) demonstrated a widened mediastinum (> 8.0cm), two (22%) showed an abnormal aortic contour, with one ( 11% ) displaying an apical cap. Confirmation of the diagnosis was obtained with either a spiral CT angiogram or transesophageal echocardiography (TEE). All nine plain radiographs were retrospectively reviewed by two board-certified radiologists aware of the diagnosis of acute dissection without a change in the readings. Conclusions Plain portable chest radiographs are of limited usefulness for the screening of acute aortic dissection. Further radiologic evaluation should be dictated by the clinical presentation and an awareness of the low sensitivity of portable chest X- rays.

  6. Endovascular Repair of Acute Uncomplicated Aortic Type B Dissection Promotes Aortic Remodelling

    DEFF Research Database (Denmark)

    Brunkwall, J; Kasprzak, P; Verhoeven, E;

    2014-01-01

    OBJECTIVES: Uncomplicated acute type B aortic dissection (AD) treated conservatively has a 10% 30-day mortality and up to 25% need intervention within 4 years. In complicated AD, stent grafts have been encouraging. The aim of the present prospective randomised trial was to compare best medical tr...

  7. Acute type B aortic dissection:update on proper management

    Institute of Scientific and Technical Information of China (English)

    Georgios Geropapas; George Galyfos; Ioannis Stefanidis; Ioannis Stamatatos; Stavros Kerasidis; Sotirios Giannakakis; Georgios Kastrisios; Gerasimos Papacharalampous; Chrisostomos Maltezos

    2014-01-01

    This study aims to collect and present all current literature data on the diagnostic and therapeutic management of acute typeB aortic dissection.It includes a comprehensive literature search utilizing the following keywords:‘acute aortic dissection’,‘typeB aortic dissection’,‘conservative management’,‘endovascular repair’,‘open surgery’ and‘diagnosis’.Uncomplicated acute type B aortic dissection can be effectively managed using conservative management, although open repair is indicated only for complicated cases.Endovascular repair shows promising results in selected patients with increased perioperative risk and without contraindications.Recent evidence supports endovascular repair even in uncomplicated cases, although more data on long-term outcomes are needed.Early risk stratification and evaluation of the patient is crucial for selection of optimal management.

  8. EXPERIENCE ON SURGICAL MANAGEMENT OF RUPTURE OF ABDOMINAL AORTIC ANEURYSM

    Institute of Scientific and Technical Information of China (English)

    管珩; 郑月宏; 李拥军; 刘昌伟; 刘暴; 叶炜

    2003-01-01

    Objective. To describe our surgical experience on rupture of abdominal aortic aneurysm .Methods. Two cases of ruptured aortic aneurysms with severe complication were analyzed. Aorta re-construction procedures were performed using bifurcated e-PTFE grafts during emergency operation. Diag-nosis, preoperative resuscitation, emergency surgical intervention, and postoperative complications of thesepatients were summarized and discussed.Results. Rupture of aortic aneurysm in both patients presented as a huge retroperitoneum haematomaby computed tomography scan. They were successfully saved by prompt body fluid compensation, emer-gency procedure, intraoperative resuscitation, and postoperative intensive care.Conclusions. Correct diagnosis, prompt surgical management, immediate intraoperative proximal aortaclamping during procedure, and effective management of postoperative complications were the key pointsto successful treatment of ruptured aortic aneurysm.

  9. Left ventricular outflow tract pseudoaneurysm formation following three aortic valve replacement surgeries

    Directory of Open Access Journals (Sweden)

    Nasrien E Ibrahim

    2015-01-01

    Full Text Available We present a case of a pseudoaneurysm arising from the left ventricular outflow tract/aortic root as a complication of aortic valve surgery. A 45-year-old Nigerian female presented to our institution′s emergency department with chest discomfort. She had three bioprosthetic aortic valve replacements in the preceding year at an outside institution for aortic regurgitation and wanted a second opinion on remaining surgical options. The learning points relevant to this case are as follows: (1 Recognizing potential complications postmultiple valve surgeries, (2 screening patients for chronic infections and rheumatologic conditions that can contribute to failed valve surgeries.

  10. Ischemic stroke secondary to aortic dissection following rifle butt recoil chest injury: a case report.

    Science.gov (United States)

    Rao, Mamatha; Panduranga, Prashanth; Al-Mukhaini, Mohammed; Al-Jufaili, Mahmood; Valiath, John

    2011-11-01

    Ischemic stroke secondary to aortic dissection is not uncommon. We present a patient with left hemiplegia secondary to Stanford type A aortic dissection extending to the supra-aortic vessels, which was precipitated by rifle butt recoil chest injury. The diagnosis of aortic dissection was delayed due to various factors. Finally, the patient underwent successful Bentall procedure with complete resolution of symptoms. This case emphasizes the need for caution in the use of firearms for recreation and to take precautions in preventing such incidents. In addition, this case illustrates the need for prompt cardiovascular physical examination in patients presenting with stroke.

  11. Balloon aortic valvuloplasty as a bridge to aortic valve surgery for severe aortic stenosis.

    Science.gov (United States)

    Nwaejike, Nnamdi; Mills, Keith; Stables, Rod; Field, Mark

    2015-03-01

    A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was, in patients with severe aortic stenosis, can balloon valvuloplasty be used as a bridge to aortic valve replacement? Altogether 463 papers were found using the reported search, of which 11 papers represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. We conclude that balloon aortic valvuloplasty is recommended as a bridge to aortic valve replacement (AVR) or transcatheter aortic valve implantation (TAVI) in patients with severe symptomatic aortic stenosis. Institutional practices, local and logistic factors can affect patient selection and management approaches to severe aortic stenosis, but having the facility to offer balloon aortic valvuloplasty (especially in the TAVI era) provides another management option for patients who would otherwise have been considered unacceptably high risk for aortic valve surgery. The increased incidence of balloon aortic valvuloplasty mirrors the increase in the use of TAVI with a sharp increase in activity from 2006. Success rates for bridging from balloon aortic valvuloplasty to definite surgical intervention are in the range 26.3-74%, with AVR or TAVI occurring within 8 weeks to 7 months. Complications from balloon aortic valvuloplasty such as aortic regurgitation (AR) can be managed successfully. Up to 40% of patients selected by balloon aortic valvuloplasty to have TAVI or AVR do not have these procedures within 2 years. While most of these patients are excluded for objective clinical reasons such as terminal disease/malignancy or other persistent contraindication, some patients refuse definitive treatment and others die while on the waiting list. Outcomes in patients bridged to AVR/TAVI are better than in patients treated with balloon aortic valvuloplasty

  12. Minimally invasive aortic valve replacement

    DEFF Research Database (Denmark)

    Foghsgaard, Signe; Schmidt, Thomas Andersen; Kjaergard, Henrik K

    2009-01-01

    In this descriptive prospective study, we evaluate the outcomes of surgery in 98 patients who were scheduled to undergo minimally invasive aortic valve replacement. These patients were compared with a group of 50 patients who underwent scheduled aortic valve replacement through a full sternotomy...... operations were completed as mini-sternotomies, 4 died later of noncardiac causes. The aortic cross-clamp and perfusion times were significantly different across all groups (P valve replacement...... is an excellent operation in selected patients, but its true advantages over conventional aortic valve replacement (other than a smaller scar) await evaluation by means of randomized clinical trial. The "extended mini-aortic valve replacement" operation, on the other hand, is a risky procedure that should...

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

    Science.gov (United States)

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

    2013-01-01

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

  14. Primary congenital abdominal aortic aneurysm: a case report with perinatal serial follow-up imaging

    International Nuclear Information System (INIS)

    Abdominal aortic aneurysms in neonates and infants are rare and are usually associated with infection, vasculitis, connective tissue disorder, or iatrogenic trauma such as umbilical catheterization. An idiopathic congenital abdominal aortic aneurysm is the least common category and there are few descriptions of the imaging features. We present the antenatal and postnatal imaging findings of an idiopathic congenital abdominal aortic aneurysm including the findings on US, MRI and CT. (orig.)

  15. Primary congenital abdominal aortic aneurysm: a case report with perinatal serial follow-up imaging

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jung Im; Lee, Whal; Chung, Jin Wook; Park, Jae Hyung [Seoul National University College of Medicine, Seoul National University Hospital, Department of Radiology, Seoul (Korea); Kim, Sang Joon [Seoul National University College of Medicine, Seoul National University Hospital, Department of Surgery, Seoul (Korea); Seo, Jeong-Wook [Seoul National University College of Medicine, Seoul National University Hospital, Department of Pathology, Seoul (Korea)

    2008-11-15

    Abdominal aortic aneurysms in neonates and infants are rare and are usually associated with infection, vasculitis, connective tissue disorder, or iatrogenic trauma such as umbilical catheterization. An idiopathic congenital abdominal aortic aneurysm is the least common category and there are few descriptions of the imaging features. We present the antenatal and postnatal imaging findings of an idiopathic congenital abdominal aortic aneurysm including the findings on US, MRI and CT. (orig.)

  16. Successful reversal of recurrent spinal cord ischemia following endovascular repair of a descending thoracic aortic aneurysm

    OpenAIRE

    Appoo, J J; Gregory, H D; Toeg, H D; Prusinkiewicz, C A; Kent, W D T; Ferland, A; Ha, D V

    2012-01-01

    Despite recent advances in technique, spinal cord ischemia remains one of the most dreaded complications of thoracic aortic surgery. Recently, it has been suggested that thoracic endovascular aortic repair may decrease the risk of paraplegia. We present a case of delayed paraplegia following thoracic endovascular aortic repair that was successfully reversed on 3 separate occasions in the same patient. This highlights the importance of vigilant clinical assessments, efficient multidisciplinary...

  17. Intestinal cholesterol embolism resulting from intra-aortic balloon pumping: a case report

    OpenAIRE

    Yamaguchi, Satoshi; Kakazu, Masanori; Osamu, Arasaki

    2014-01-01

    Introduction Intra-aortic balloon pumping is used in elective percutaneous coronary intervention for increasing coronary blood flow. However, intra-aortic balloon pumping may decrease visceral blood flow and cause mesenteric ischemia by visceral artery obstruction. Case presentation We report the case of a 79-year-old Asian man in whom elective percutaneous coronary intervention was performed with intra-aortic balloon pumping. He died from mesenteric ischemia 25 hours after the procedure. Mic...

  18. Valve-sparing aortic root replacement and aortic valve repair in a patient with acromegaly and aortic root dilatation

    Directory of Open Access Journals (Sweden)

    Karel Van Praet

    2015-07-01

    Full Text Available Aortic regurgitation and dilatation of the aortic root and ascending aorta are severe complications of acromegaly. The current trend for management of an aortic root aneurysm is valve-sparing root replacement as well as restoring the diameter of the aortic sinotubular junction (STJ and annulus. Our case report supports the recommendation that in patients with acromegaly, severe aortic root involvement may indicate the need for surgery.

  19. Abdominal aortic aneurysm surgery

    DEFF Research Database (Denmark)

    Gefke, K; Schroeder, T V; Thisted, B;

    1994-01-01

    The goal of this study was to identify patients who need longer care in the ICU (more than 48 hours) following abdominal aortic aneurysm (AAA) surgery and to evaluate the influence of perioperative complications on short- and long-term survival and quality of life. AAA surgery was performed in 553......, 78% stated that their quality of life had improved or was unchanged after surgery and had resumed working. These data justify a therapeutically aggressive approach, including ICU therapy following AAA surgery, despite failure of one or more organ systems....

  20. Unoperated aortic aneurysm

    DEFF Research Database (Denmark)

    Perko, M J; Nørgaard, M; Herzog, T M;

    1995-01-01

    . No significant differences in survival for patients with dissecting and nondissecting AA were detected. In all, 132 patients (78%) died and 78 (59%) of them died of rupture. Mean time to rupture was 1,300 +/- 8 days. Cumulative 5-year hazard of rupture for the dissecting AA was twice that of the nondissecting (p......From 1984 to 1993, 1,053 patients were admitted with aortic aneurysm (AA) and 170 (15%) were not operated on. The most frequent reason for nonoperative management was presumed technical inoperability. Survivals for patients with thoracic, thoracoabdominal, and abdominal AA were comparable...

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

    Science.gov (United States)

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

    2016-04-01

    Almost one-quarter of patients presenting with thoracic aortic aneurysms (TAAs) or acute aortic dissections (TAADs) have an underlying mutation in a specific gene. A subset of these patients will have systemic syndromic features, for example, skeletal features in patients with Marfan Syndrome. It is important to note that the majority of patients with thoracic aortic disease will not have these syndromic features but many will have a family history of the disease. The genes predisposing to these thoracic aortic diseases are inherited in an autosomal dominant manner, and thirteen genes have been identified to date. As the clinical phenotype associated with each specific gene is defined, the data indicate that the underlying gene dictates associated syndromic features. More importantly, the underlying gene also dictates the aortic disease presentation, the risk for dissection at a given range of aortic diameters, the risk for additional vascular diseases and what specific vascular diseases occur associated with the gene. These results lead to the recommendation that the medical and surgical management of these patients be dictated by the underlying gene, and for patients with mutations in ACTA2, the specific mutation in the gene. PMID:26837258

  2. Aortic biomechanics in hypertrophic cardiomyopathy

    Science.gov (United States)

    Badran, Hala Mahfouz; Soltan, Ghada; Faheem, Nagla; Elnoamany, Mohamed Fahmy; Tawfik, Mohamed; Yacoub, Magdi

    2015-01-01

    Background: Ventricular-vascular coupling is an important phenomenon in many cardiovascular diseases. The association between aortic mechanical dysfunction and left ventricular (LV) dysfunction is well characterized in many disease entities, but no data are available on how these changes are related in hypertrophic cardiomyopathy (HCM). Aim of the work: This study examined whether HCM alone is associated with an impaired aortic mechanical function in patients without cardiovascular risk factors and the relation of these changes, if any, to LV deformation and cardiac phenotype. Methods: 141 patients with HCM were recruited and compared to 66 age- and sex-matched healthy subjects as control group. Pulse pressure, aortic strain, stiffness and distensibility were calculated from the aortic diameters measured by M-mode echocardiography and blood pressure obtained by sphygmomanometer. Aortic wall systolic and diastolic velocities were measured using pulsed wave Doppler tissue imaging (DTI). Cardiac assessment included geometric parameters and myocardial deformation (strain and strain rate) and mechanical dyssynchrony. Results: The pulsatile change in the aortic diameter, distensibility and aortic wall systolic velocity (AWS') were significantly decreased and aortic stiffness index was increased in HCM compared to control (P < .001) In HCM AWS' was inversely correlated to age(r = − .32, P < .0001), MWT (r = − .22, P < .008), LVMI (r = − .20, P < .02), E/Ea (r = − .16, P < .03) LVOT gradient (r = − 19, P < .02) and severity of mitral regurg (r = − .18, P < .03) but not to the concealed LV deformation abnormalities or mechanical dyssynchrony. On multivariate analysis, the key determinant of aortic stiffness was LV mass index and LVOT obstruction while the role LV dysfunction in aortic stiffness is not evident in this population. Conclusion: HCM is associated with abnormal aortic mechanical properties. The severity of cardiac

  3. Abdominal aortic grafting for spontaneous infrarenal abdominal aortic dissection.

    Science.gov (United States)

    Iwasaki, Hiroto; Shibuya, Takashi; Shintani, Takashi; Uenaka, Hisazumi; Suehiro, Shigefumi; Satoh, Hisashi

    2010-02-01

    This case report concerns a 62-year-old woman with spontaneous infrarenal abdominal aortic dissection, which developed into claudication and rest pain in the lower extremity. Multi-row detector computed tomography showed the entry site of the abdominal aortic dissection at the second lumbar artery, while the reentry site was found intraoperatively at the median sacral artery, indicating that the false lumen had progressed and compressed the true lumen. A direct approach involving grafting appears to be an effective procedure for resolving mesenteric and lower extremity hypoperfusion due to aortic dissection with a dilated false channel, even during the acute period. PMID:19879731

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

    Directory of Open Access Journals (Sweden)

    Han Qing-qi

    2013-06-01

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

  5. Acute aortic dissection: be aware of misdiagnosis

    Directory of Open Access Journals (Sweden)

    Asteri Theodora

    2009-02-01

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

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

    Science.gov (United States)

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

    2016-05-01

    We describe the case of a 28-year old man with Marfan's syndrome and severe pectus excavatum who required an aortic root replacement for an ascending aortic aneurysm. There was a near-vertical angulation of the sternum that presented challenges with opening and exposure of the heart during aortic surgery. Furthermore, removal of the sternal retractor after aortic repair resulted in sudden loss of cardiac output. A Ravitch procedure was then performed to successfully close the chest without further cardiovascular compromise. We propose that patients with a severe pectus excavatum and mediastinal displacement seen on preoperative CT scanning should be considered for simultaneous, elective repair. PMID:26874148

  7. [Pannus Formation Two Years after Bioprosthetic Aortic Valve Implantation;Report of a Case].

    Science.gov (United States)

    Ono, Kimiyo; Kuroda, Hiroaki

    2015-08-01

    We report a case of early deterioration of the bioprosthetic aortic valve 23 months postoperatively. A 77-year-old man who had undergone aortic valve replacement with a 23-mm Epic valve( St. Jude Medical [SJM])presented to us after a syncopal episode. Echocardiography revealed severe aortic stenosis, and redo aortic valve replacement with a 21-mm SJM mechanical valve was performed. All 3 cusps of the tissue valve were thickened by fibrous pannus overgrowth. Neither calcification nor invasion of inflammatory cells was observed. The cause of pannus formation at such an early stage after implantation remains unknown. PMID:26329714

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

    Science.gov (United States)

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

    2016-05-01

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

  9. Glucose Suppresses Biological Ferroelectricity in Aortic Elastin

    Science.gov (United States)

    Liu, Yuanming; Wang, Yunjie; Chow, Ming-Jay; Chen, Nataly Q.; Ma, Feiyue; Zhang, Yanhang; Li, Jiangyu

    2013-04-01

    Elastin is an intriguing extracellular matrix protein present in all connective tissues of vertebrates, rendering essential elasticity to connective tissues subjected to repeated physiological stresses. Using piezoresponse force microscopy, we show that the polarity of aortic elastin is switchable by an electrical field, which may be associated with the recently discovered biological ferroelectricity in the aorta. More interestingly, it is discovered that the switching in aortic elastin is largely suppressed by glucose treatment, which appears to freeze the internal asymmetric polar structures of elastin, making it much harder to switch, or suppressing the switching completely. Such loss of ferroelectricity could have important physiological and pathological implications from aging to arteriosclerosis that are closely related to glycation of elastin.

  10. Spontaneous aortic dissecting hematoma in two dogs.

    Science.gov (United States)

    Boulineau, Theresa Marie; Andrews-Jones, Lydia; Van Alstine, William

    2005-09-01

    This report describes 2 cases of spontaneous aortic dissecting hematoma in young Border Collie and Border Collie crossbred dogs. Histology was performed in one of the cases involving an unusual splitting of the elastin present within the wall of the aorta, consistent with elastin dysplasia as described in Marfan syndrome in humans. The first case involved a young purebred Border Collie that died suddenly and the second case involved a Border Collie crossbred dog that died after a 1-month history of seizures. Gross lesions included pericardial tamponade with dissection of the ascending aorta in the former case and thoracic cavity hemorrhage, mediastinal hematoma, and aortic dissection in the latter. Histologic lesions in the case of the Border Collie crossbred dog included a dissecting hematoma of the ascending aorta with elastin dysplasia and right axillary arterial intimal proliferation. PMID:16312247

  11. Assessment of the influence of the compliant aortic root on aortic valve mechanics by means of a geometrical model.

    Science.gov (United States)

    Redaelli, A; Di Martino, E; Gamba, A; Procopio, A M; Fumero, R

    1997-12-01

    In recent years several researchers have suggested that the changes in the geometry and angular dimensions of the aortic root which occur during the cardiac cycle are functional to the optimisation of aortic valve function, both in terms of diminishing leaflet stresses and of fluid-dynamic behaviour. The paper presents an analytical parametric model of the aortic valve which includes the aortic root movement. The indexes used to evaluate the valve behaviour are the circumferential membrane stress and the stress at the free edge of the leaflet, the index of bending strain, the bending of the leaflet at the line attachment in the radial and circumferential directions and the shape of the conduit formed by the leaflets during systole. In order to evaluate the role of geometric changes in valve performance, two control cases were considered, with different reference geometric configuration, where the movement of the aortic root was ignored. The results obtained appear consistent with physiological data, especially with regard to the late diastolic phase and the early ejection phase, and put in evidence the role of the aortic root movement in the improvement of valve behaviour. PMID:9450254

  12. Has percutaneous aortic valve replacement taken center stage in the treatment of aortic valve disease?

    Science.gov (United States)

    Kumar, Gideon Praveen; Cui, Fangsen; Mathew, Lazar; Leo, Hwa Liang

    2013-01-01

    Modern biomedical advances have propelled percutaneous valve replacement into an effective and powerful therapy for many heart valve diseases, especially aortic valve stenosis. Experiences so far suggest that outcomes for new percutaneous valve replacement surgery compare favorably with that of traditional valve surgery in selected patients with severe symptomatic aortic stenosis. The inception of percutaneous aortic valve replacement (PAVR) began in 1992 when the potential for treating valve diseases was demonstrated through a modern technique of endoluminal deployment of a catheter-mounted crimped stented heart valve in an animal model. The first successful demonstration of such novel technique of surgical replacement of a heart valve was performed in 2002, when valve implantation in a patient with aortic stenosis was reported. Despite initial stumbles and a perception of being an uphill task, PAVR has emerged as one of the breakthroughs in surgical procedures. More than 1500 citations were found in PubMed, half of which were available after 2011. This is primarily because more than 50,000 procedures are being performed in more than 40 countries worldwide, with encouraging outcomes, and several stented valves have been launched in the market. This review provides a detailed analysis of the current state of the art of PAVR. Moreover, a competitive landscape of various devices available in the market and their design considerations, biomaterial selections, and overall hemodynamic performance are presented. PMID:24941416

  13. Extended aortic valvuloplasty: a new approach for the management of congenital valvar aortic stenosis.

    Science.gov (United States)

    Ilbawi, M N; DeLeon, S Y; Wilson, W R; Roberson, D A; Husayni, T S; Quinones, J A; Arcilla, R A

    1991-09-01

    A new technique for the treatment of congenital valvar aortic stenosis is described. It consists of augmenting the aortic cusp by extending the commissurotomy incision into the aortic wall around the leaflet insertion, mobilizing the valve cusp attachment at the commissures, and freeing the aortic insertion of the rudimentary commissure. The results of standard valvotomy performed on 48 patients (group 1) were compared with those of the new extended valvuloplasty carried out on 16 patients (group 2). The two groups were comparable in age at operation (2.7 +/- 2.1 years for group 1 versus 2.1 +/- 1.7 years for group 2; p = not significant) and in preoperative pressure gradient (58 +/- 25 mm Hg for group 1 versus 61 +/- 36 mm Hg for group 2; p = not significant). There was no operative mortality in either group. Follow-up is available on all patients, with a mean of 4.3 +/- 2.6 years for group 1 versus 1.7 +/- 0.5 years for group 2 (p = 0.05). There was one late death in group 1. Postoperative gradient was 47 +/- 13 mm Hg in group 1 versus 19 +/- 13 mm Hg in group 2 (p = 0.05). Moderate or severe regurgitation was present in 18 patients (38%) in group 1 and 2 patients (13%) in group 2 (p = not significant). Reoperation was needed in 8 patients (17%) in group 1 versus 2 patients (13%) in group 2 (p = not significant). The described valvuloplasty procedure addresses the unique pathological features of valvar aortic stenosis and provides better relief of the obstruction than the presently available techniques. Longer follow-up is needed to determine the late results of this approach.

  14. Regional hypoplasia of somatosensory cortex in growth-retarded mice (grt/grt).

    Science.gov (United States)

    Sawada, Kazuhiko; Saito, Shigeyoshi; Sugasawa, Akari; Sato, Chika; Aoyama, Junya; Ohara, Naoko; Horiuchi-Hirose, Miwa; Kobayashi, Tetsuya

    2016-07-01

    Growth-retarded mouse (grt/grt) is a spontaneous mutant that is known as an animal model for primary congenital hypothyroidism caused by resistance to TSH signaling. The regional pattern of cerebral cortical hypoplasia was characterized in grt/grt mice. Ex vivo computed tomography (CT)-based volumetry was examined in four regions of the cerebral cortex, i.e., prefrontal, frontal, parietal and occipito-temporal regions, which were demarcated by structural landmarks on coronal CT images. A region-specific reduced volume of the parietal cortical region covering most of the somatosensory cortex was noted in grt/grt mice rather than in both heterozygous (grt/+) and wild-type (+/+) mice. We concluded that the cortical hypoplasia in grt/grt was seen in identical cortical regions corresponding to human congenital hypothyroidism. PMID:26915353

  15. A case of superior segmental optic hypoplasia accompanied by a glaucomatous optic neuropathy

    OpenAIRE

    Ohguro, Hiroshi

    2008-01-01

    Ikuyo Ohguro, Hiroshi OhguroDepartment of Ophthalmology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, JapanAbstract: This is the first case report of a bilateral superior segmental optic hypoplasia (SSOH) accompanied by a glaucomatous optic neuropathy (GON). A 47-year-old man incidentally diagnosed as having bilateral SSOH, simultaneously disclosed glaucomatous optic disc appearances, including enlargements of the cup of the optic nerve heads and a thinning of the infero-...

  16. Interproximal contact hypoplasia in primary teeth: A new enamel defect with anthropological and clinical relevance.

    Science.gov (United States)

    Lukacs, John R.

    1999-11-01

    This study reports the prevalence, distribution, and expression of enamel defects in a sample of primary teeth (n = 225) from a prehistoric site in western India (1400-700 BC). Five enamel surfaces of individual, isolated primary teeth were observed for surface defects using a binocular stereomicroscope with variable power of magnification (8-20x). Standards for evaluating dental enamel defects (DDE) recommended by the Fédération Dentaire International (FDI) were employed. Details of defect expression were also recorded, including size, shape, and surface of tooth crown affected. Hypoplastic enamel defects were observed in 28% of teeth, but the distribution and expression of defects was not random. More than 50% of canine teeth had hypoplastic defects (HD); incisors and molar teeth exhibited far fewer HD. The buccal surface of canines was the most commonly affected crown surface. Areas of missing enamel were also common on the mesial and distal surfaces of canines and incisors and on the mesial surface of molar teeth. The high frequency of enamel defects found on interproximal crown surfaces warrants a label, and the name interproximal contact hypoplasia (IPCH) is proposed. Linear enamel hypoplasia (LEH) was absent from this primary dental sample. IPCH is more frequent in mandibular than in maxillary teeth, but no side preference was detected. In canine teeth, buccal hypoplasias (localized hypoplasia of primary canines; LHPC) were not positively correlated with interproximal hypoplastic defects. The etiology of IPCH may involve mesial compaction of developing teeth due to slow longitudinal growth of the jaws. Episodic bone remodeling results in ephemeral fenestrae in the mesial and distal walls of the dental crypt permitting tooth-tooth contact and disruption of amelogenesis. IPCH prevalence decreases across the subsistence transition from sedentary Early Jorwe agriculturalists to seminomadic Late Jorwe hunters and foragers, but the difference is not

  17. Neuropathological features in a female fetus with OPHN1 deletion and cerebellar hypoplasia.

    Science.gov (United States)

    Rocas, Delphine; Alix, Eudeline; Michel, Jessica; Cordier, Marie-Pierre; Labalme, Audrey; Guilbert, Hélène; Till, Marianne; Schluth-Bolard, Caroline; de Haas, Pascale; Massardier, Jérôme; Portes, Vincent des; Edery, Patrick; Touraine, Renaud; Guibaud, Laurent; Vasiljevic, Alexandre; Sanlaville, Damien

    2013-05-01

    We report the case of a 33-year-old pregnant woman. The third-trimester ultrasound scan during pregnancy revealed fetal bilateral ventricular dilatation, macrosomia and a transverse diameter of the cerebellum at the 30th centile. A brain MRI scan at 31 weeks of gestation led to a diagnosis of hypoplasia of the cerebellar vermis without hemisphere abnormalities and a non compressive expansion of the cisterna magna. The fetal karyotype was 46,XX. The pregnancy was terminated and array-CGH analysis of the fetus identified a 238 kb de novo deletion on chromosome Xp12, encompassing part of OPHN1 gene. Further studies revealed a completely skewed pattern of X inactivation. OPHN1 is involved in X-linked mental retardation (XLMR) with cerebellar hypoplasia and encodes a Rho-GTPase-activating protein called oligophrenin-1, which is produced throughout the developing mouse brain and in the hippocampus and Purkinje cells of the cerebellum in adult mice. Neuropathological examination of the female fetus revealed cerebellar hypoplasia and the heterotopia of Purkinje cells at multiple sites in the white matter of the cerebellum. This condition mostly affects male fetuses in humans. We report here the first case of a de novo partial deletion of OPHN1, with radiological and neuropathological examination, in a female fetus. PMID:23416624

  18. [Risk factors for teeth aplasia and hypoplasia in cleft lip and palate children].

    Science.gov (United States)

    Korolenkova, M V; Starikova, N V; Ageeva, L V

    2016-01-01

    The aim of the study was to assess the significance of environmental risk factors for teeth aplasia and hypoplasia in cleft lip and palate children. Two hundred and forty-seven cleft lip and palate (CLP) children were enrolled in the study including 105 (42.5%) with bilateral CLP and 57.5% with unilateral CLP. The mean age was 11.2±4.9 years. Teeth condition was assessed clinically and radiologically. The impact of risk factors for teeth anomalies was analyzed by retrospective data obtained from computer database (absence of preoperative orthopedic treatment, palatal defects after primary palatoplasty and type of primary procedures). Surgical trauma by early periosteoplasty (at the age of 3-4 months), excessive scarring and tissue traction due to absence of early orthopedic treatment and palatal defect were associated with significantly higher incidence of incisors hypoplasia (both developmental enamel defects and microdentia) and aplasia of central incisors not seen in the other study subgroups. Incisors aplasia and hypoplasia in CLP patients do not always have disembryogenic origin but may depend on external environmental factors, including surgical trauma.

  19. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... see we've used the cautery to achieve access to the sternum. On the left hand side ... wound, which allows us to get more ready access to the aorta and the aortic valve. The ...

  20. Transcatheter Aortic Heart Valve Thrombosis

    DEFF Research Database (Denmark)

    Hansson, Nicolaj C; Grove, Erik L; Andersen, Henning R;

    2016-01-01

    BACKGROUND: There is increasing focus on transcatheter heart valve (THV) thrombosis. However, there are limited data on incidence, clinical implications and predisposing factors of THV thrombosis following transcatheter aortic valve replacement (TAVR). OBJECTIVES: We assessed the incidence...

  1. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... No medications as such have been proven to slow that disease path life. Again, it's a progressive ... no medical therapy that has been proven to slow or reverse the process of aortic stenosis. Clearly, ...

  2. [ENDOVASCULAR ABDOMINAL AORTIC ANEURISM REPAIR].

    Science.gov (United States)

    Maĭstrenko, D N; Generalov, M I; Tarazov, P G; Zherebtsov, F K; Osovskikh, V V; Ivanov, A S; Oleshchuk, A N; Granov, D A

    2015-01-01

    The authors analyzed the single-center experience of treatment of 72 patients with abdominal aortic aneurisms and severe accompanied pathology. The aneurisms were repaired by stent-grafts. All the patients had abdominal aortic aneurisms with the diameters from 41 to 84 mm against the background of severe somatic pathology. It was a contraindication to planned open surgery. An installation of stent-graft was successful in all 72 follow-ups. It wasn't necessary to use a conversion to open surgery. The follow-up period consisted of 44,6?2,1 months. Control ultrasound and computer tomography studies hadn't revealed an increase of aneurism sack sizes or "eakages". A reduction of abdominal aortic aneurism sizes was noted in 37 patients on 4-5% during first year after operation. The stent-graft implantation extends the possibilities of abdominal aortic aneurism treatment for patients from a high surgical risk group. PMID:26234059

  3. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... to minimize their symptoms, but that doesn't impact the course of the disease itself. When I' ... more likely we see aortic stenosis. Again, patient education is part of the evaluation and management of ...

  4. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... for patients who would not tolerate well a traditional open operation or a less invasive operation, as ... physical reserve. So Barbara Bush recently had a traditional aortic valve replacement surgery. What makes a patient ...

  5. Adult thoracic and abdominal aortic

    Directory of Open Access Journals (Sweden)

    Randa O. Kaddah

    2016-06-01

    Conclusion: Aortic COA could be found in any segment of the aorta. Proper identification of the anatomical details and pressure gradient studies are important factors affecting the plan of management.

  6. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... symptoms and ultimately surgery may be in the future. The symptoms seen primarily with aortic stenosis at ... echocardiography is? Echocardiography is the use of ultrasound technology. Ultrasound technology is a form of the same ...

  7. CONGENITAL QUADRICUSPID AORTIC-VALVE

    NARCIS (Netherlands)

    BROUWER, MHJ; DEGRAAF, JJ; EBELS, T

    1993-01-01

    Two patients with a quadricuspid aortic valve are described, one of them with concomitant juxtaposed coronary orifices facing the right hand facing sinus. The etiology and incidence of this congenital anomaly will be discussed.

  8. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... somebody for this operation. Again, there's no medical therapy that has been proven to slow or reverse ... to their physician. This may lead to earlier treatment and better outcomes as well. Aortic stenosis, as ...

  9. Volumetric analysis of abdominal aortic aneurysm

    Science.gov (United States)

    Baskin, Kevin M.; Kusnick, Catherine A.; Shamsolkottabi, Susanne; Lang, Elvira V.; Corson, J. D.; Stanford, William; Thompson, Brad H.; Hoffman, Eric A.

    1996-04-01

    The purpose of this study was to develop a valid, reliable and accurate system of measurement of abdominal aortic aneurysms, using volumetric analysis of x-ray computed tomographic data. This study evaluates illustrative cases, and compares measurements of AAA phantoms, using standard 2D versus volumetric methods. To validate the volumetric analysis, four phantom aneurysms were constructed in a range of diameters (4.5 - 7.0 cm) which presents the greatest management challenge to the clinician. These phantoms were imaged using a Toshiba Xpress SX helical CT. Separate scans were obtained at conventional (10 mm X 10 mm) and thin slice (5 mm X 5 mm) collimations. The thin slices were reconstructed at 2 mm intervals. Data from each of the 96 scans were interpreted using a standard 2D approach, then analyzed using task-oriented volumetric software. We evaluate patient assessments, and compare greatest outer diameters of phantoms, by standard versus volumetric methods. Qualitative differences between solutions based on standard versus volumetric analysis of illustrative patient cases are substantial. Expert radiologists' standard measurements of phantom aneurysms are highly reliable (r2 equals 0.901 - 0.958; p aneurysm diameters in the range of clinical interest. For the same phantoms, volumetric analysis was both more reliable (r2 equals 0.986 - 0.996; p abdominal aortic aneurysms. It is particularly valuable in the presence of aortic tortuosity, vessel eccentricity, and uncertain involvement of critical vessels.

  10. Cadaveric aorta implantation for aortic graft infection.

    Science.gov (United States)

    Ali, Asad; Bahia, Sandeep S S; Ali, Tahir

    2016-01-01

    This case report describes a 73-year-old gentleman who underwent explantation of an infected prosthetic aorto-iliac graft and replacement with a cryopreserved thoracic and aorto-iliac allograft. The patient has been followed up a for more than a year after surgery and remains well. After elective tube graft repair of his abdominal aortic aneurysm (AAA) in 2003, he presented to our unit in 2012 in cardiac arrest as a result of a rupture of the distal graft suture line due to infection. After resuscitation he underwent aorto-bifemoral grafting using a cuff of the original aortic graft proximally. Distally the new graft was anastomosed to his common femoral arteries, with gentamicin beads left in situ. Post discharge the patient was kept under close surveillance with serial investigations including nuclear scanning, however it became apparent that his new graft was infected and that he would require aortic graft replacement, an operation with a mortality of at least 50%. The patient underwent the operation and findings confirmed a synthetic graft infection. This tube graft was explanted and a cryopreserved aorta was used to the refashion the abdominal aorta and its bifurcation. The operation required a return to theatre day one post operatively for a bleeding side branch, which was repaired. The patient went on to make a full recovery stepping down from the intensive therapy unit day 6 post operatively and went on to be discharged 32 days after his cryopreserved aorta implantation. PMID:27351624

  11. Characteristics of aortic valve dysfunction and ascending aorta dimensions according to bicuspid aortic valve morphology

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Hong Ju [Konkuk University Medical Center, Konkuk University School of Medicine, Department of Cardiovascular Surgery, Seoul (Korea, Republic of); Yonsei University College of Medicine, Department of Cardiovascular Surgery, Seoul (Korea, Republic of); Shin, Je Kyoun; Chee, Hyun Kun; Kim, Jun Suk [Konkuk University Medical Center, Konkuk University School of Medicine, Department of Cardiovascular Surgery, Seoul (Korea, Republic of); Ko, Sung Min [Konkuk University Medical Center, Konkuk University School of Medicine, Department of Radiology, Seoul (Korea, Republic of)

    2015-07-15

    To characterize aortic valve dysfunction and ascending aorta dimensions according to bicuspid aortic valve (BAV) morphology using computed tomography (CT) and surgical findings. We retrospectively enrolled 209 patients with BAVs who underwent transthoracic echocardiography (TTE) and CT. BAVs were classified as anterior-posterior (BAV-AP) or lateral (BAV-LA) orientation of the cusps and divided according to the presence (raphe+) or absence (raphe-) of a raphe. Ascending aortic dimensions were measured by CT at four levels. BAV-AP was present in 129 patients (61.7 %) and raphe+ in 120 (57.4 %). Sixty-nine patients (33.0 %) had aortic regurgitation (AR), 70 (33.5 %) had aortic stenosis (AS), and 58 (27.8 %) had combined AS and AR. AR was more common in patients with BAV-AP and raphe+; AS was more common with BAV-LA and raphe-.Annulus/body surface area and tubular portion/body surface area diameters in patients with BAV-AP (17.1 ± 2.3 mm/m{sup 2} and 24.2 ± 5.3 mm/m{sup 2}, respectively) and raphe+ (17.3 ± 2.2 mm/m{sup 2} and 24.2 ± 5.5 mm/m{sup 2}, respectively) were significantly different from those with BAV-LA (15.8 ± 1.9 mm/m{sup 2} and 26.4 ± 5.5 mm/m{sup 2}, respectively) and raphe- (15.7 ± 1.9 mm/m{sup 2} and 26.2 ± 5.4 mm/m{sup 2}, respectively). The morphological characteristics of BAV might be associated with the type of valvular dysfunction, and degree and location of an ascending aorta dilatation. (orig.)

  12. Endovascular treatment of aortic pathologies - State of the art. Part 1 - Aneurysms of abdominal aorta

    International Nuclear Information System (INIS)

    Endovascular treatment of aortic pathologies is actually an alternative to open surgery. It has proven to be safe, showing similar or better results to those achieved by surgery. In this article, treatment of aortic pathologies by means of endoprosthesis is presented, as well as its indications, contraindications and future treatment with this kind of devices

  13. Aortic stiffness is associated with atherosclerosis of the coronary arteries in older adults : the Rotterdam Study

    NARCIS (Netherlands)

    van Popele, Nicole M.; Mattace-Raso, Francesco U. S.; Vliegenthart, Rozemarijn; Grobbee, Diederick E.; Asmar, Roland; van der Kuip, Deirdre A. M.; Hofman, Albert; de Feijter, Pim J.; Oudkerk, Matthijs; Witteman, Jacqueline C. M.

    2006-01-01

    Objective Aortic stiffness can lead to low diastolic blood pressure, thereby possibly limiting coronary perfusion. Therefore, the simultaneous occurrence of both aortic stiffness and coronary atherosclerosis can lead to an increased risk of subendocardial ischaemia. The aim of the present study was

  14. Aortic stiffness is associated with atherosclerosis of the coronary arteries in older adults: the Rotterdam Study

    NARCIS (Netherlands)

    van Popele, N.M.; Mattace-Raso, F.U.S.; Vliegenthart, R.; Grobbee, D.E.; Asmar, R.; van der Kuip, D.A.M.; Hofman, A.; de Feijter, P.J.; Oudkerk, M.; Witteman, J.C.M.

    2006-01-01

    Objective Aortic stiffness can lead to low diastolic blood pressure, thereby possibly limiting coronary perfusion. Therefore, the simultaneous occurrence of both aortic stiffness and coronary atherosclerosis can lead to an increased risk of subendocardial ischaemia. The aim of the present study was

  15. Staged and hybrid approach in the treatment of complex aortic dissection

    Institute of Scientific and Technical Information of China (English)

    WANG Li-xin; FU Wei-guo; GUO Da-qiao; CHEN Bin; JIANG Jun-hao; YANG Jue; SHI Zhen-yu; WANG Yu-qi

    2008-01-01

    @@ Multilevel aortic disease presents a formidable challenge for vascular surgeons. In the past, multilevel aortic surgery was performed simultaneously or subsequently. Single-stage intervention is thought to be associated with a high incidence of complications, and sequential repair requires several major surgical interventions.

  16. Iliac branched device implantation in tortuous iliac anatomy after previous open ruptured aortic aneurysm repair

    NARCIS (Netherlands)

    Vourliotakis, G.; Bracale, U. M.; Sondakh, A.; Tielliu, I. F. J.; Prins, T. R.; Verhoeven, E. L. G.

    2012-01-01

    The aim of this paper was to present iliac branched device (IBD) implantation in a fit 67-year-old man with tortuous iliac anatomy after previous emergent open abdominal aortic aneurysm (AAA) repair. The patient underwent open treatment for a ruptured abdominal aortic aneurysm in another hospital. T

  17. Endograft failure in an adult patient with coarctation and bicuspid aortic valve.

    Science.gov (United States)

    Dimarakis, Ioannis; Grant, Stuart; Kadir, Isaac

    2013-06-01

    A 25-year-old man presented with associated bicuspid aortic valve and coarctation of the aorta. Following aortic valve replacement, he underwent endovascular stenting of his native coarctation. We describe early failure of the latter procedure that necessitated definitive surgical correction.

  18. Enamel hypoplasia in the deciduous teeth of great apes: variation in prevalence and timing of defects.

    Science.gov (United States)

    Lukacs, J R

    2001-11-01

    The prevalence of enamel hypoplasia in the deciduous teeth of great apes has the potential to reveal episodes of physiological stress in early stages of ontogenetic development. However, little is known about enamel defects of deciduous teeth in great apes. Unresolved questions addressed in this study are: Do hypoplastic enamel defects occur with equal frequency in different groups of great apes? Are enamel hypoplasias more prevalent in the deciduous teeth of male or female apes? During what phase of dental development do enamel defects tend to form? And, what part of the dental crown is most commonly affected? To answer these questions, infant and juvenile skulls of two sympatric genera of great apes (Gorilla and Pan) were examined for dental enamel hypoplasias. Specimens from the Powell-Cotton Museum (Quex Park, UK; n = 107) are reported here, and compared with prior findings based on my examination of juvenile apes at the Cleveland Museum of Natural History (Hamman-Todd Collection; n = 100) and Smithsonian Institution (National Museum of Natural History; n = 36). All deciduous teeth were examined by the author with a x10 hand lens, in oblique incandescent light. Defects were classified using Fédération Dentaire International (FDI)/Defects of Dental Enamel (DDE) standards; defect size and location on the tooth crown were measured and marked on dental outline charts. Enamel defects of ape deciduous teeth are most common on the labial surface of canine teeth. While deciduous incisor and molar teeth consistently exhibit similar defects with prevalences of approximately 10%, canines average between 70-75%. Position of enamel defects on the canine crown was analyzed by dividing it into three zones (apical, middle, and cervical) and calculating defect prevalence by zone. Among gorillas, enamel hypoplasia prevalence increases progressively from the apical zone (low) to the middle zone to the cervical zone (highest), in both maxillary and mandibular canine teeth

  19. 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

    Full Text Available OBJETIVO: Estudar a viabilidade técnica e resultados imediatos da modificação técnica proposta por Caliani et al. para correção da coarctação aórtica com hipoplasia do arco aórtico. MÉTODOS: Entre janeiro de 2005 e julho de 2006, nove neonatos com coarctação aórtica e hipoplasia do arco aórtico foram submetidos a uma nova abordagem cirúrgica para correção do defeito. A definição de hipoplasia do arco aórtico seguiu os critérios de Moulaert, segundo os quais o arco aórtico é considerado hipoplásico quando seu diâmetro atinge 50% do diâmetro da aorta ascendente. Nesta série, foram selecionados apenas pacientes com hipoplasia proximal e distal do arco aórtico. Várias técnicas foram propostas anteriormente, mas gradientes residuais importantes foram observados, assim como há o inconveniente da ligadura definitiva da artéria subclávia esquerda. A modificação técnica consiste em: toracotomia póstero-lateral esquerda, ampla mobilização da aorta descendente, com ligadura dos dois primeiros ramos intercostais, transecção da artéria subclávia esquerda em sua base, ressecção ampla de toda zona hipoplásica e adjacências do ducto arterioso, anastomose término-terminal entre o arco aórtico e aorta descendente com fio de PDS 7-0 e reimplante da artéria subclávia sobre a artéria carótida esquerda com anastomose látero-terminal. RESULTADOS: Não houve óbito per-operatório ou tardio, o gradiente residual médio foi de 5 mmHg. Até o presente, não observamos nenhum caso de recoarctação ou de lesão neurológica medular. CONCLUSÃO: A despeito do reduzido número de casos e do seguimento curto, esta modificação técnica pode representar uma excelente opção para tratamento deste complexo grupo de pacientes.OBJECTIVES: To study technical feasibility and early results of the technical modification suggested by Caliani et al. for correction of aortic coarctation and aortic arc. METHODS: Between January

  20. A case report of focal dermal hypoplasia-Goltz syndrome

    Directory of Open Access Journals (Sweden)

    Sheela Bharani

    2013-01-01

    Full Text Available Goltz syndrome is a rare multisystem disorder with cutaneous, ocular, dental and skeletal abnormalities. Other mesoectodermal abnormalities are also present. Its hallmark is thinning of the dermis resulting subcutaneous fat herniation. The present case is a 5 year old girl having linear skin atrophy with fat herniation, skeletal abnormalities in the form of polysyndactyly, facial asymmetry, squint with coloboma iris, deformed pinna, abnormal dentition, umbilical hernia along with osteopathia striata of long bones which is consistent with Goltz syndrome. We are presenting this case due to its rarity.

  1. A case report of focal dermal hypoplasia-Goltz syndrome.

    Science.gov (United States)

    Bharani, Sheela; Thakkar, Sejal

    2013-07-01

    Goltz syndrome is a rare multisystem disorder with cutaneous, ocular, dental and skeletal abnormalities. Other mesoectodermal abnormalities are also present. Its hallmark is thinning of the dermis resulting subcutaneous fat herniation. The present case is a 5 year old girl having linear skin atrophy with fat herniation, skeletal abnormalities in the form of polysyndactyly, facial asymmetry, squint with coloboma iris, deformed pinna, abnormal dentition, umbilical hernia along with osteopathia striata of long bones which is consistent with Goltz syndrome. We are presenting this case due to its rarity. PMID:23984248

  2. A case report of focal dermal hypoplasia-Goltz syndrome

    OpenAIRE

    Sheela Bharani; Sejal Thakkar

    2013-01-01

    Goltz syndrome is a rare multisystem disorder with cutaneous, ocular, dental and skeletal abnormalities. Other mesoectodermal abnormalities are also present. Its hallmark is thinning of the dermis resulting subcutaneous fat herniation. The present case is a 5 year old girl having linear skin atrophy with fat herniation, skeletal abnormalities in the form of polysyndactyly, facial asymmetry, squint with coloboma iris, deformed pinna, abnormal dentition, umbilical hernia along with osteopathia ...

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

    Science.gov (United States)

    Omeje, Ikenna; Ram, Awat; Kostolny, Martin

    2013-02-01

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

  4. Salmonella aortitis treated with endovascular aortic repair: a case report

    Directory of Open Access Journals (Sweden)

    Strahm Carol

    2012-08-01

    Full Text Available Abstract Introduction Salmonella is a typical cause of aortitis, which is associated with high morbidity and mortality. In infrarenal disease, besides open surgery, endovascular aortic repair as an alternative treatment has been reported. To the best of our knowledge, we report the first successful endovascular aortic repair documented by necropsy to date. Case presentation A 67-year-old Caucasian man presented with low back pain, fever and positive blood cultures for Salmonella Enteritidis. A computed tomography scan showed an enlargement and intramural hematoma of the infrarenal aortic wall; a Salmonella aortitis was suspected and antimicrobial therapy initiated. Because of substantial comorbidities, endovascular aortic repair was favored over open surgery; postoperatively the antibiotic treatment was continued for 12 months. Post-mortem there were neither macroscopic nor microscopic signs of aortitis or graft infection. Conclusions We could demonstrate by necropsy that endovascular aortic repair of infrarenal aortitis with prolonged pre- and postinterventional antibiotic therapy for 12 months was a minimally invasive alternative and should be considered in selected clinically stable patients with substantial co-morbidities.

  5. Gene expression signature of cerebellar hypoplasia in a mouse model of Down syndrome during postnatal development

    Directory of Open Access Journals (Sweden)

    Vitalis Tania

    2009-03-01

    population that is thought responsible for the cerebellar hypoplasia in Down syndrome, a global destabilization of gene expression was not detected. Altogether these results strongly suggest that the three-copy genes are directly responsible for the phenotype present in cerebellum. We provide here a short list of candidate genes.

  6. Aneurismas da aorta Aortic aneurysms

    Directory of Open Access Journals (Sweden)

    Januário M Souza

    1992-09-01

    Full Text Available Entre janeiro de 1979 e janeiro de 1992, foram realizadas 212 operações para correção de aneurismas e de dissecções da aorta. Neste trabalho serão analisados 104 procedimentos cirúrgicos (em 97 pacientes para correção de aneurismas. A idade dos pacientes variou de 14 a 79 anos (média 59,5 anos e o sexo predominante foi o masculino, com 75 pacientes. Os aneurismas localizavam-se na aorta ascendente em 46 pacientes, na croça em 8, na aorta descendente em 8, na aorta toráco-abdominal em 8, na aorta abdominal em 21, na aorta descendente e abdominal em 2, na aorta ascendente e tóraco-abdominal em 2, na aorta ascendente e descendente em 1, na aorta ascendente, croça e descendente em 1. Doenças cardiovasculares associadas estavam presentes em 39 pacientes, sendo valvopatia aórtica em 18 (excluídos os pacientes com ectasiaânulo-aórtíca, insuficiência coronária em 17, coarctação da aorta em 2, persistência do canal arterial em 1 e valvopatia mitral e aórtica em 1. A mortalidade imediata (hospitalar e/ou 30 dias foi de 14,4%, sendo de 27,7% (5/18 para pacientes com mais de 70 anos e de 11,3% (9/79 para pacientes com idade inferior a 70 anos. Os aneurismas localizados na aorta ascendente e croça foram operados como o auxílio de circulação extracorpórea. Parada circulatória e hipotermia profunda foram utilizadas em todos os pacientes com aneurisma da croça. O estudo tomográfico e angiográfico deve ser de toda a aorta, pela possibilidade de aneurismas de localizações múltiplas.Among 212 patients undergoing operation for aortic aneurysm and aortic dissection between January 1979 and January 1992, 97 were operated on for aneurysms. The aneurysms were localized in: ascending aorta in 46 patients, transverse aortic arch in 8, descending aorta in 8, thoracoabdominal aorta in 8, abdominal (infrarenal aorta in 21, descending and abdominal aorta in 2, ascending and thoracoabdominal aorta in 2, ascending and descending in 1

  7. Fractured cervical spine and aortic transection.

    LENUS (Irish Health Repository)

    Griffin, M J

    2012-02-03

    A 17-year-old victim of a road traffic accident presented. Following investigation diagnoses of fractured first cervical vertebra, aortic transection, diffuse cerebral oedema, fractured right ribs 2-4 and pubic rami were made. Management of this case presented a number of anaesthetic dilemmas: management of the airway, use of cross-clamp vs. shunting or heparinization and bypass, cardiovascular and neurological monitoring, maintenance of cardiovascular stability during and post cross-clamp, minimizing the risk of post-operative renal and neurological dysfunction.

  8. Acute aortic valve prolapse in Marfan's syndrome.

    OpenAIRE

    Carr, N J; Cullen, S. A.

    1991-01-01

    A 22 year old man with Marfan's syndrome died suddenly following acute aortic valve prolapse. Although aortic root involvement in Marfan's syndrome is common, we have found no previous description of this particular complication in the literature.

  9. A planning system for transapical aortic valve implantation

    Science.gov (United States)

    Gessat, Michael; Merk, Denis R.; Falk, Volkmar; Walther, Thomas; Jacobs, Stefan; Nöttling, Alois; Burgert, Oliver

    2009-02-01

    Stenosis of the aortic valve is a common cardiac disease. It is usually corrected surgically by replacing the valve with a mechanical or biological prosthesis. Transapical aortic valve implantation is an experimental minimally invasive surgical technique that is applied to patients with high operative risk to avoid pulmonary arrest. A stented biological prosthesis is mounted on a catheter. Through small incisions in the fifth intercostal space and the apex of the heart, the catheter is positioned under flouroscopy in the aortic root. The stent is expanded and unfolds the valve which is thereby implanted into the aortic root. Exact targeting is crucial, since major complications can arise from a misplaced valve. Planning software for the perioperative use is presented that allows for selection of the best fitting implant and calculation of the safe target area for that implant. The software uses contrast enhanced perioperative DynaCT images acquired under rapid pacing. In a semiautomatic process, a surface segmentation of the aortic root is created. User selected anatomical landmarks are used to calculate the geometric constraints for the size and position of the implant. The software is integrated into a PACS network based on DICOM communication to query and receive the images and implants templates from a PACS server. The planning results can be exported to the same server and from there can be rertieved by an intraoperative catheter guidance device.

  10. [Late complication of surgical repair of aortic coarctation: ruptured pseudoaneurysm of the aorta treated by thoracic endovascular aortic repair].

    Science.gov (United States)

    Varejka, P; Lubanda, J C; Prochazka, P; Heller, S; Beran, S; Dostal, O; Charvat, F; Horejs, J; Semrad, M; Linhart, A

    2010-06-01

    Aortic coarctation is a frequent congenital defect requiring early surgical treatment. Late complications of these surgical procedures can be fatal as in the case of a ruptured anastomotic pseudoaneurysm. We present a case of a 49-year-old man presenting with hemorrhagic shock due to this complication who was successfully treated by endovascular techniques with implantation of two stent grafts. This case illustrates the fact that endovascular aortic repair is feasible, certainly less invasive and very efficient for this type of complication when used in an experienced center.

  11. ABDOMINAL AORTIC ANEURYSM (AAA

    Directory of Open Access Journals (Sweden)

    Rajesh G

    2011-11-01

    Full Text Available AAA is defined as a distension of the infrarenal aorta by more than 50% (or 1.5 times compared with a corresponding healthy, aged and gender matched population. AAA afflicts 1 to 6 % of the general population aged more than 60 years and the incidence rises by approximately 0.15% annually. When the definition of a maximum external diameter ≥3 cm is used, the prevalence of AAA is upto 6 times greater in men than in women. AAAs are much more common than thoracic aortic aneurysms. Most common cause of AAA is atherosclerosis (95%. Less common causes include infectious or inflammatory origin or those associated with connective tissue disorders. Process of AAA formation is multifactorial. Other than the general risk factors for atherosclerosis, genetic predispo s iti on, aut o immunity and hemodynamic factors all play roles in its formation. AAA is 1.5 times more frequent in hypert ensive patients. Smokers have 8 times risk for developing AAA. The existence of familial aggre gation of AAA implicates genetic factors in the etiology of AAA. Women with AAA are more likely to have a positive family history of this disorder. Those with a family history of AAA have an increased risk of 30%, and their aneurysms tend to occur at a younger age and carry a greater risk of rupture than do sporadic aneurysms.

  12. Open aortic surgery after thoracic endovascular aortic repair.

    Science.gov (United States)

    Coselli, Joseph S; Spiliotopoulos, Konstantinos; Preventza, Ourania; de la Cruz, Kim I; Amarasekara, Hiruni; Green, Susan Y

    2016-08-01

    In the last decade, thoracic endovascular aortic aneurysm repair (TEVAR) has emerged as an appealing alternative to the traditional open aortic aneurysm repair. This is largely due to generally improved early outcomes associated with TEVAR, including lower perioperative mortality and morbidity. However, it is relatively common for patients who undergo TEVAR to need a secondary intervention. In select circumstances, these secondary interventions are performed as an open procedure. Although it is difficult to assess the rate of open repairs after TEVAR, the rates in large series of TEVAR cases (>300) have ranged from 0.4 to 7.9 %. Major complications of TEVAR that typically necessitates open distal aortic repair (i.e., repair of the descending thoracic or thoracoabdominal aorta) include endoleak (especially type I), aortic fistula, endograft infection, device collapse or migration, and continued expansion of the aneurysm sac. Conversion to open repair of the distal aorta may be either elective (as for many endoleaks) or emergent (as for rupture, retrograde complicated dissection, malperfusion, and endograft infection). In addition, in select patients (e.g., those with a chronic aortic dissection), unrepaired sections of the aorta may progressively dilate, resulting in the need for multiple distal aortic repairs. Open repairs after TEVAR can be broadly classified as full extraction, partial extraction, or full salvage of the stent-graft. Although full and partial stent-graft extraction imply failure of TEVAR, such failure is generally absent in cases where the stent-graft can be fully salvaged. We review the literature regarding open repair after TEVAR and highlight operative strategies.

  13. Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Disease

    DEFF Research Database (Denmark)

    Mylotte, Darren; Lefevre, Thierry; Søndergaard, Lars;

    2014-01-01

    BACKGROUND: Limited information exists describing the results of transcatheter aortic valve (TAV) replacement in patients with bicuspid aortic valve (BAV) disease (TAV-in-BAV). OBJECTIVES: This study sought to evaluate clinical outcomes of a large cohort of patients undergoing TAV-in-BAV. METHODS......: We retrospectively collected baseline characteristics, procedural data, and clinical follow-up findings from 12 centers in Europe and Canada that had performed TAV-in-BAV. RESULTS: A total of 139 patients underwent TAV-in-BAV with the balloon-expandable transcatheter heart valve (THV) (n = 48...

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1991-01-01

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

  15. The everyday used nomenclature of the aortic root components: the tower of Babel?

    Science.gov (United States)

    Sievers, Hans-Hinrich; Hemmer, Wolfgang; Beyersdorf, Friedhelm; Moritz, Anton; Moosdorf, Rainer; Lichtenberg, Artur; Misfeld, Martin; Charitos, Efstratios I

    2012-03-01

    Modern analyses of data for scientific reporting and healthcare management purposes require standardized and consistent definitions, something which also holds true for aortic root surgery, as part of the cardiovascular surgery spectrum. The aim of the present study was to investigate the currently employed nomenclature of the aortic root components. A questionnaire was constructed on the terminology of aortic root components, providing a list of common definitions including anatomical descriptions, as well as fields for custom responses. Responses were received from 534 cardiothoracic surgeons registered at www.ctsnet.org. Remarkable variations in definitions were detected. The most unanimously accepted terms were: 'aortic leaflets', the freely moving parts (52.6% of responses); 'commissures', the distal part of the leaflet attachments plus the peripheral area of the free edges of the leaflets (52.2%); 'semi-lunar leaflet attachment', the anatomic site of leaflet attachment (58%); 'annulus', the circular line defined by the nadirs of the leaflets (38%); 'interleaflet triangle', the tissue between two leaflets and annulus (23%); 'aortic valve', the three leaflets only (55%); 'aortic root' as composed of sinuses, tissue between the leaflets, sinutubular junction, leaflets and their wall attachment (63%). The remarkable variability on the everyday-used definitions of the aortic root components can potentially lead to misinterpretation of data. More stringent adoption of consistent, standardized definitions of aortic root components is necessary in the modern era of data collection and management. PMID:22345173

  16. Simulation of bifurcated stent grafts to treat abdominal aortic aneurysms (AAA)

    CERN Document Server

    Egger, Jan; Freisleben, Bernd

    2016-01-01

    In this paper a method is introduced, to visualize bifurcated stent grafts in CT-Data. The aim is to improve therapy planning for minimal invasive treatment of abdominal aortic aneurysms (AAA). Due to precise measurement of the abdominal aortic aneurysm and exact simulation of the bifurcated stent graft, physicians are supported in choosing a suitable stent prior to an intervention. The presented method can be used to measure the dimensions of the abdominal aortic aneurysm as well as simulate a bifurcated stent graft. Both of these procedures are based on a preceding segmentation and skeletonization of the aortic, right and left iliac. Using these centerlines (aortic, right and left iliac) a bifurcated initial stent is constructed. Through the implementation of an ACM method the initial stent is fit iteratively to the vessel walls - due to the influence of external forces (distance- as well as balloonforce). Following the fitting process, the crucial values for choosing a bifurcated stent graft are measured, ...

  17. Isolated femoral hypoplasia: an intrauterine differential diagnosis to campomelia

    Energy Technology Data Exchange (ETDEWEB)

    Koerber, Friederike; Benz-Bohm, Gabriele [University of Cologne, Department of Paediatric Radiology, Cologne (Germany); Schoenau, Eckard [University of Cologne, Department of Paediatrics, Cologne (Germany); Horwitz, A.Eldad [Klinikum Krefeld, Department of Paediatric Radiology, Krefeld (Germany)

    2005-06-01

    The isolated form of femoral bowing is an important differential diagnosis of campomelia. Therefore, knowledge of isolated anomalies is fundamental for prenatal diagnosis, especially for the differential diagnosis from severe syndromes. Four cases are presented to discuss the differential diagnosis of femoral bowing including a review of the literature. We report four newborn babies with unilateral bowing and shortening of the femur. Three had no further anomaly; one child had additional abnormalities due to coumarin embryopathy. The radiological findings were shortened femora with bowing and varus deformity and cortical thickening on the concave side. All other parts showed normal bone structure. The aetiology of femoral bowing is unknown. Early damage of the cartilaginous model followed by remodelling with thickening on the concave side of the bone similar to the healing of malaligned fractures is suspected. The isolated form of femoral bowing without any other anomalies has to be differentiated from complex and more often severe congenital syndromes such as campomelia. Postpartum radiological examination should be reduced to a single exposure of the affected limb and follow-up should be done by clinical examination. (orig.)

  18. Shape-based diagnosis of the aortic valve

    Science.gov (United States)

    Ionasec, Razvan Ioan; Tsymbal, Alexey; Vitanovski, Dime; Georgescu, Bogdan; Zhou, S. Kevin; Navab, Nassir; Comaniciu, Dorin

    2009-02-01

    Disorders of the aortic valve represent a common cardiovascular disease and an important public-health problem worldwide. Pathological valves are currently determined from 2D images through elaborate qualitative evalu- ations and complex measurements, potentially inaccurate and tedious to acquire. This paper presents a novel diagnostic method, which identies diseased valves based on 3D geometrical models constructed from volumetric data. A parametric model, which includes relevant anatomic landmarks as well as the aortic root and lea ets, represents the morphology of the aortic valve. Recently developed robust segmentation methods are applied to estimate the patient specic model parameters from end-diastolic cardiac CT volumes. A discriminative distance function, learned from equivalence constraints in the product space of shape coordinates, determines the corresponding pathology class based on the shape information encoded by the model. Experiments on a heterogeneous set of 63 patients aected by various diseases demonstrated the performance of our method with 94% correctly classied valves.

  19. Metastatic neuroendocrine carcinoma of aortic body origin in a cat.

    Science.gov (United States)

    Hansen, Sonya C; Smith, Annette N; Kuo, Kendon W; Fish, Eric J; Koehler, Jey W; Martinez-Romero, Gisela; Bacek, Lenore M

    2016-09-01

    An 8-year-old, female spayed Domestic Shorthair cat was presented to the Auburn University Emergency and Critical Care service for evaluation of pleural effusion and a suspected intrathoracic mass. Computed tomography was performed which confirmed the presence of a large intrathoracic mass, likely heart-based. Fine-needle aspirates were obtained and a cytologic diagnosis of a neuroendocrine tumor was made. Treatment with toceranib phosphate was briefly attempted at home by the owners. The cat died at home approximately 6 weeks after diagnosis. Necropsy and subsequent histopathologic examination revealed a metastatic neuroendocrine carcinoma of aortic body origin. Aortic body tumors are extremely rare in cats and to the authors' knowledge, a neuroendocrine carcinoma of aortic body origin with distant metastases has not yet been reported in a cat. PMID:27564688

  20. Aortic dissection accompanied by preeclampsia in a postpartum young woman

    Science.gov (United States)

    Park, Jin-Wan; Kim, Su-Mi; Yu, Gyu-Bong

    2016-01-01

    Aortic dissection is very rare in obstetrics, but it is a fatal disease. A 37-weeks primigravida woman with dyspnea and pitting edema presented to our emergency room. The patient was diagnosed with preeclampsia and underwent an emergency cesarean section under spinal anesthesia. The patient complained of severe dyspnea after the cesarean section, and the chest computed tomography scan was done. With the finding of aortic dissection, cardiopulmonary arrest occurred 5 hours after the cesarean section, and the patient died without reaction to cardio-pulmonary resuscitation. If a patient with preeclampsia complains of severe dyspnea or chest pain, aortic dissection needs to be suspected and a diagnosis should not be delayed. PMID:27668205

  1. Acute myocardial ischemia after aortic valve replacement: A comprehensive diagnostic evaluation using dynamic multislice spiral computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Lembcke, Alexander [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany)]. E-mail: alexander.lembcke@gmx.de; Hein, Patrick A. [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany); Enzweiler, Christian N.H. [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany); Hoffmann, Udo [Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States); Klessen, Christian [Department of Radiology, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany); Dohmen, Pascal M. [Department of Cardiovascular Surgery, Charite-Universitaetsmedizin Berlin, Freie Universitaet Berlin and Humboldt-Universitaet zu Berlin, Berlin (Germany)

    2006-03-15

    We describe the case of a 72-year-old man presenting with endocarditis and clinical signs of acute myocardial ischemia after biological aortic valve replacement. A comprehensive cardiac dynamic multislice spiral computed tomography demonstrated: (1) an endocarditic vegetation of the aortic valve; (2) a subvalvular leakage feeding a paravalvular pseudoaneurysm based on an aortic root abscess with subsequent compromise of the systolic blood flow in the left main coronary artery and the resulting myocardial perfusion deficit.

  2. [MINIMALLY INVASIVE AORTIC VALVE REPLACEMENT].

    Science.gov (United States)

    Tabata, Minoru

    2016-03-01

    Minimally invasive aortic valve replacement (MIAVR) is defined as aortic valve replacement avoiding full sternotomy. Common approaches include a partial sternotomy right thoracotomy, and a parasternal approach. MIAVR has been shown to have advantages over conventional AVR such as shorter length of stay and smaller amount of blood transfusion and better cosmesis. However, it is also known to have disadvantages such as longer cardiopulmonary bypass and aortic cross-clamp times and potential complications related to peripheral cannulation. Appropriate patient selection is very important. Since the procedure is more complex than conventional AVR, more intensive teamwork in the operating room is essential. Additionally, a team approach during postoperative management is critical to maximize the benefits of MIAVR. PMID:27295772

  3. [MINIMALLY INVASIVE AORTIC VALVE REPLACEMENT].

    Science.gov (United States)

    Tabata, Minoru

    2016-03-01

    Minimally invasive aortic valve replacement (MIAVR) is defined as aortic valve replacement avoiding full sternotomy. Common approaches include a partial sternotomy right thoracotomy, and a parasternal approach. MIAVR has been shown to have advantages over conventional AVR such as shorter length of stay and smaller amount of blood transfusion and better cosmesis. However, it is also known to have disadvantages such as longer cardiopulmonary bypass and aortic cross-clamp times and potential complications related to peripheral cannulation. Appropriate patient selection is very important. Since the procedure is more complex than conventional AVR, more intensive teamwork in the operating room is essential. Additionally, a team approach during postoperative management is critical to maximize the benefits of MIAVR.

  4. A Child with Lung Hypoplasia, Congenital Heart Disease, Hemifacial Microsomia, and Inguinal Hernia: Ipsilateral Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Chengming Fan

    2015-01-01

    Full Text Available A 3-year-old Chinese boy was diagnosed with ipsilateral congenital malformations: right lung hypoplasia, dextroversion of heart, atrial septal defect, hepatic vein drainage directly into the right atrium, facial asymmetry, right microtia and congenital deafness, and indirect inguinal hernia. He underwent indirect inguinal hernia repair at the age of 2. Although without any facial plastic surgery performed, he underwent a repair of atrial septal defect and recovered uneventfully. At 6-month follow-up, the patient was free from any symptom of dyspnea; his heart function returned to the first grade.

  5. [Aplasia and hypoplasia of the side of the nose in the Mocha Period and today].

    Science.gov (United States)

    Pirsig, W

    1989-01-01

    In earthenware vessels from the Moche period (200-700 A.D.) pathological findings of nasal deformities have been depicted in a realistic manner. By means of two recent casuistics and of two portrait vessels of Old Peru it is demonstrated how exactly the nasal pathology was observed by the artists of the Moche period. The first example shows a unilateral hypoplasia of the nasal ala due to injury in early childhood, the second example shows a bilateral aplasia of the nasal alae due to an intrauterine growth disturbance. PMID:2690796

  6. Weight reduction and aortic stiffness in obese children and adolescents

    DEFF Research Database (Denmark)

    Hvidt, K. N.; Olsen, M. H.; Ibsen, H.;

    2015-01-01

    Little is known about the effect of weight reduction on aortic stiffness and especially so in the young. The present study investigates whether weight reduction influences aortic stiffness in obese children and adolescents. Carotid-femoral pulse wave velocity (cfPWV) and augmentation index at heart...... rate 75 (AIx@HR75) were measured in 72 obese patients aged 10-18 years at baseline and after 1-year of lifestyle intervention (follow-up). We found that although the degree of obesity decreased (Delta body mass index z-score: -0.24 +/- 0.45, P ....27 +/- 0.47 ms(-1), P obesity measures. No significant change...

  7. Chaotic musical murmur in aortic regurgitation.

    Science.gov (United States)

    Miyahara, K; Amitani, S; Sohara, H; Kurose, M; Iwamura, H; Toyohira, H; Taira, A

    1996-12-01

    We report an interesting case of aortic regurgitation. Phonocardiographically, the shape of the diastolic musical murmur in this case changed in each cardiac cycle despite being in sinus rhythm, in the same posture and in the same breathing phase. Experimentally, we were able to obtain a similar noise pattern using an artificial respirator and a hemispherical silicone membrane. We concluded that the irregular and chaotic change in the shape of the diastolic musical murmur in the present case occurred due to irregular swaying of the non-coronary cusp under the influence of the Venturi effect owing to a regurgitant jet stream.

  8. Percutaneous Implantation of the self-expanding valve Prosthesis a patient with homozygous familial hypercholesterolemia severe aortic stenosis and porcelain aorta.

    Science.gov (United States)

    Sahiner, Levent; Asil, Serkan; Kaya, Ergün Baris; Ozer, Necla; Aytemir, Kudret

    2016-10-01

    Transcatheter aortic valve implantation (TAVI) has shown favorable outcomes in patients with severe symptomatic aortic valve stenosis who are at high surgical risk or inappropriate for open heart surgery. However, concerns exist over treating patients who have porcelain aorta and familial hypercholesterolemia, due to the potential complications of aortic root and aortic annulus. In this case report, we present a patient with familial hypercholesterolemia, symptomatic severe aortic stenosis, previous coronary artery bypass grafting and porcelain aorta, who was successfully treated with TAVI using a CoreValve. PMID:27393846

  9. Trends in pediatric ostomy surgery: intestinal diversion for necrotizing enterocolitis and biliary diversion for biliary hypoplasia syndromes.

    Science.gov (United States)

    Bastawrous, A A; Torosian, M B; Statter, M B; Arensman, R M

    1995-11-01

    Ostomies are placed in children for different indications than in the older population. Many ostomies of childhood are placed because of congenital or neonatal problems that require temporary or long-term diversion to stabilize the neonatal patient. Necrotizing enterocolitis, the most common reason for placement of neonatal colostomies and ileostomies, is increasing in frequency as more prematurely born infants survive. Recently, there has been an increase in treatment of various biliary hypoplasia syndromes with biliary cutaneous diversion. Children with biliary hypoplasia syndromes are a challenging group of patients who frequently can be helped by ostomies. This article reviews current information on biliary cutaneous diversion for the biliary hypoplasia syndromes and intestinal diversion for necrotizing enterocolitis.

  10. Transfemoral Valve-in-Valve Transcatheter Aortic Valve Implantation (TAVI) in a Patient With Previous Endovascular Aortic Repair (EVAR).

    Science.gov (United States)

    Ruparelia, Neil; Panoulas, Vasileios F; Frame, Angela; Nathan, Anthony W; Ariff, Ben; Jaffer, Usman; Sutaria, Nilesh; Chukwuemeka, Andrew; Mikhail, Ghada W; Malik, Iqbal S

    2016-07-01

    A 90-year-old man presented with increasing exertional breathlessness. He had previous implantation of a Perimount bioprosthetic aortic valve (Edwards Lifesciences) and coronary artery bypass graft surgery. Due to severe transvalvular bioprosthetic regurgitation with preserved left ventricular dimensions and ejection fraction, the heart team decided on valve-in- valve transcatheter aortic valve implantation via the transfemoral route in view of the patient's prohibitively high surgical and anesthetic risk. The patient had an uncomplicated recovery and was symptomatically much improved at 3-month follow-up. PMID:27342209

  11. Using The Descending Aortic Wall Thickness Measured In Transesophageal Echocardiography As A Risk Marker For Aortic Dissection

    OpenAIRE

    Zaher Fanari; Sumaya Hammami; Muhammad Baraa Hammami; Safa Hammami; Chete Eze-Nliam; Weintraub, William S.

    2015-01-01

    Objective: The aim of this study is to estimate whether aortic wall thickness is increased in patients with Aortic dissection (AD) compared to low risk control group and can be used in addition to aortic diameter as a risk marker of AD. Background: AD occurs due to pathologies that may increase thickness of the aortic wall. Transesophageal echocardiography (TEE) has the ability to visualise both the thoracic aortic wall and lumen. Aortic diameter has been used to predict aortic dissection...

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

    Science.gov (United States)

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

    2014-01-01

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

  13. Renal embolism as a primary manifestation of Streptococcus dysgalactiae subspecies equisimilis endocarditis in a patient with chronic aortic dissection.

    Science.gov (United States)

    Ishimaru, Naoto; Kinami, Saori; Ohnishi, Hisashi; Takagi, Asuka; Kawamoto, Megumi; Doukuni, Ryota; Umezawa, Kanoko; Oozone, Sachiko; Yoshimura, Sho; Sakamoto, Susumu

    2015-06-01

    We report a case of renal embolism as an initial manifestation of Streptococcus dysgalactiae subspecies equisimilis (SDSE) endocarditis in a patient with chronic aortic dissection. A 37-year-old man who underwent total aortic arch replacement owing to aortic dissection, presented with a 3-h history of fever, chills, and acute right-sided flank pain. The endocarditis affected the native aortic valve and was complicated by a renal embolism. Blood culture results were positive for SDSE. Intravenous penicillin resulted in satisfactory clinical and echocardiographic recovery. PMID:26110298

  14. Deep gluteal grounding pad burn after abdominal aortic aneurysm repair.

    Science.gov (United States)

    Sapienza, Paolo; Venturini, Luigi; Cigna, Emanuele; Sterpetti, Antonio V; Biacchi, Daniele; di Marzo, Luca

    2015-01-01

    Although skin burns at the site of grounding pad are a known risk of surgery, their exact incidence is unknown. We first report the case of a patient who presented a deep gluteal burn at the site of the grounding pad after an abdominal aortic aneurism repair, the etiology and the challenging treatment required to overcome this complication. PMID:26099000

  15. [A hybrid approach to surgery for thoracic aortic aneurysm

    DEFF Research Database (Denmark)

    L., de la Motte; Baekgaard, N.; Jensen, L.P.;

    2009-01-01

    A 57-year-old male, previously treated surgically with insertion of grafts for type A and B aortic dissection, presented with a pulsatile mass in the jugular fossa. Further examination verified a pseudoaneurysm the inlet of which was located at the proximal anastomotic site of the descending aort...

  16. Aortic bifurcation tear following blunt trauma in childhood

    OpenAIRE

    Shlomo Yellinek; Dimitri Gimelrich; Ofer Merin; Petachia Reissman; Marc Arkovitz

    2015-01-01

    Rupture of the abdominal aorta from blunt trauma is rare and aortic biforcation tear is extremely rare. We will present the management of a 2 year old boy who suffered blunt abdominal trauma and was operated in urgent fashion in our institution.

  17. Case report of Streptomyces endocarditis of a prosthetic aortic valve.

    Science.gov (United States)

    Mossad, S B; Tomford, J W; Stewart, R; Ratliff, N B; Hall, G S

    1995-01-01

    We describe the first case of prosthetic valve endocarditis due to a Streptomyces sp. The patient presented with fever, cutaneous embolic lesions, and bacteremia 3 months after aortic valve replacement. Treatment required valve replacement and a long course of parenteral imipenem. PMID:8586732

  18. Case report of Streptomyces endocarditis of a prosthetic aortic valve.

    OpenAIRE

    Mossad, S B; Tomford, J W; Stewart, R; Ratliff, N B; Hall, G. S.

    1995-01-01

    We describe the first case of prosthetic valve endocarditis due to a Streptomyces sp. The patient presented with fever, cutaneous embolic lesions, and bacteremia 3 months after aortic valve replacement. Treatment required valve replacement and a long course of parenteral imipenem.

  19. Transcatheter, valve-in-valve transapical aortic and mitral valve implantation, in a high risk patient with aortic and mitral prosthetic valve stenoses

    Directory of Open Access Journals (Sweden)

    Harish Ramakrishna

    2015-01-01

    Full Text Available Transcatheter valve implantation continues to grow worldwide and has been used principally for the nonsurgical management of native aortic valvular disease-as a potentially less invasive method of valve replacement in high-risk and inoperable patients with severe aortic valve stenosis. Given the burden of valvular heart disease in the general population and the increasing numbers of patients who have had previous valve operations, we are now seeing a growing number of high-risk patients presenting with prosthetic valve stenosis, who are not potential surgical candidates. For this high-risk subset transcatheter valve delivery may be the only option. Here, we present an inoperable patient with severe, prosthetic valve aortic and mitral stenosis who was successfully treated with a trans catheter based approach, with a valve-in-valve implantation procedure of both aortic and mitral valves.

  20. [Aortic inflammatory lesions in Behçet's disease].

    Science.gov (United States)

    Desbois, A-C; Wechsler, B; Cacoub, P; Saadoun, D

    2016-04-01

    The arterial lesions affect about 10% of patients with Behçet's disease (BD). Aortic inflammatory involvement includes predominantly aortic aneurysmal lesions affecting most often the abdominal aorta. They account for the severity of the disease and are a leading cause of death when they hit the aorta or pulmonary arteries. Within the arterial lesions of BD, aortic involvement is, with femoral lesions, the most common site involved (18-28% of patients with vascular disease). Unlike other large vessels vasculitis (i.e. giant cell arteritis and Takayasu's arteritis) diffuse aortitis is observed in less than 5% of patients with BD. Aortic lesions of BD may be asymptomatic (systematic imaging or occasionally associated with other vascular event) or be revealed by the occurrence of abdominal, thoracic or lumbar pain, or an aortic valve insufficiency. Fever is frequently associated. Increase in acute phase reactants is common in these patients. Histological analysis may show infiltration by lymphocytes, neutrophils and plasma cells in the media and adventitia and a proliferation of the vasa vasorum in the media as well as a fibroblastic proliferation. In the later phase, a fibrous thickening of the media and adventitia is observed as well as a proliferation and thickening of the vasa vasorum. The therapeutic management should always include a medical treatment for the control of inflammation (corticosteroids, immunosuppressive drugs and/or biotherapy) and often an endovascular or surgical treatment if the aneurysm is threatening. The choice between endovascular or surgical treatment is considered case by case, depending on the experience of the team, anatomical conditions and of the clinical presentation. In this review, we provide a detailed and updated review of the literature to describe the aortic inflammatory damage associated with Behçet's disease. PMID:26611428

  1. Restoration of incisal half with edge-up technique using ceramic partial crown in turner's hypoplasia: A case report.

    Science.gov (United States)

    Hegde, Shreya; Kundabala, M

    2014-01-01

    This case report describes a rare treatment modality for Turner's hypoplasia done with a very conservative approach for the esthetic and functional problem of the defect. Diagnosis was made as Turner's hypoplasia of upper two central incisors with proximal caries. Treatment planning was done after considering many factors such as conservation of tooth structure, esthetics, occlusion and economy. Tooth preparation was done to receive Edge-up, all ceramic partial crowns for both the upper central incisors,using pressable all ceramic material and cemented with resin cement. PMID:24554869

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

    Science.gov (United States)

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

    2016-03-01

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

  3. Type F Congenital Quadricuspid Aortic Valve: A Very Rare Case Diagnosed by 3-dimenional Transoesophageal Echocardiography

    Science.gov (United States)

    Garg, Pankaj; Kamaruddin, Hazlyna; Orme, Rachel; Watt, Victoria

    2014-01-01

    Congenital quadricuspid aortic valve (QAV) is a rare cardiac anomaly. Several different anatomical variations of a quadricuspid aortic valve have been described. Aortic regurgitation is the predominant valvular dysfunction associated with QAV and patients tend to present in their 5th or 6th decade of life. This anomaly is rarely picked up by transthoracic echocardiogram (TTE). A comprehensive transoesophageal echocardiography (TOE) study is more likely to diagnose it. We describe a very rare type of QAV – Type F in a 52-year-old lady who presented with symptoms of shortness of breath and pre-syncope. We include TOE images and intra-operative valve images. PMID:24707324

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

    Directory of Open Access Journals (Sweden)

    Hager Alfred

    2010-03-01

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

  5. Spectrum of pontocerebellar hypoplasia in 13 girls and boys with CASK mutations: confirmation of a recognizable phenotype and first description of a male mosaic patient

    Directory of Open Access Journals (Sweden)

    Burglen Lydie

    2012-03-01

    Full Text Available Abstract Background Pontocerebellar hypoplasia (PCH is a heterogeneous group of diseases characterized by lack of development and/or early neurodegeneration of cerebellum and brainstem. According to clinical features, seven subtypes of PCH have been described, PCH type 2 related to TSEN54 mutations being the most frequent. PCH is most often autosomal recessive though de novo anomalies in the X-linked gene CASK have recently been identified in patients, mostly females, presenting with intellectual disability, microcephaly and PCH (MICPCH. Methods Fourteen patients (12 females and two males; aged 16 months-14 years presenting with PCH at neuroimaging and with clinical characteristics unsuggestive of PCH1 or PCH2 were included. The CASK gene screening was performed using Array-CGH and sequencing. Clinical and neuroradiological features were collected. Results We observed a high frequency of patients with a CASK mutation (13/14. Ten patients (8 girls and 2 boys had intragenic mutations and three female patients had a Xp11.4 submicroscopic deletion including the CASK gene. All were de novo mutations. Phenotype was variable in severity but highly similar among the 11 girls and was characterized by psychomotor retardation, severe intellectual disability, progressive microcephaly, dystonia, mild dysmorphism, and scoliosis. Other signs were frequently associated, such as growth retardation, ophthalmologic anomalies (glaucoma, megalocornea and optic atrophy, deafness and epilepsy. As expected in an X-linked disease manifesting mainly in females, the boy hemizygous for a splice mutation had a very severe phenotype with nearly no development and refractory epilepsy. We described a mild phenotype in a boy with a mosaic truncating mutation. We found some degree of correlation between severity of the vermis hypoplasia and clinical phenotype. Conclusion This study describes a new series of PCH female patients with CASK inactivating mutations and confirms that

  6. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... more likely we see aortic stenosis. Again, patient education is part of the evaluation and management of ... want to embrace less invasive or more progressive technology, you want to make ... with that information and certainly shouldn't be insulted by that. ...

  7. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... well. I want to underscore again that exercise testing is really not recommended in patient with classic symptoms in severe aortic stenosis, as it's probably more stress than the patient's heart should have to undergo. Once again, serial evaluations, as with any chronic disease process, are ...

  8. Correlations of perioperative coagulopathy, fluid infusion and blood transfusions with survival prognosis in endovascular aortic repair for ruptured abdominal aortic aneurysm

    OpenAIRE

    Kawatani, Yohei; Nakamura, Yoshitsugu; Kurobe, Hirotsugu; Suda, Yuji; Hori, Takaki

    2016-01-01

    Background Factors associated with survival prognosis among patients who undergo endovascular aortic repair (EVAR) for ruptured abdominal aortic aneurysms (rAAA) have not been sufficiently investigated. In the present study, we examined correlations between perioperative coagulopathy and 24-h and 30-day postoperative survival. Relationships between coagulopathy and the content of blood transfusions, volumes of crystalloid infusion and survival. Methods This was a retrospective study of the me...

  9. Grading system modification and management of blunt aortic injury

    Institute of Scientific and Technical Information of China (English)

    Kaavya N Reddy; Tim Matatov; Linda D Doucet; Maureen Heldmann; Cynthia X Zhao; Wayne W Zhang

    2013-01-01

    Background The traditional approach to blunt aortic injury (BAI) has been emergent intervention.This study aimed to utilize a modified imaging grading system that may allow us to categorize these injuries as needing emergent,urgent,or non-operative management.Methods From January 2003 to December 2011,28 patients with BAI were managed at our institution.Imaging and medical records were reviewed retrospectively.BAI was classified into 4 grades based on imaging studies.Grade la:intimal tear,Grade Ⅰb:intramural hematoma; Grade Ⅱ:intimal injury with periaotic hematoma; Grade lia:aortic transection with pseudoaneurysm,Grade Ⅲb:multiple aortic injuries; and Grade Ⅳ:free rupture.Progression and clinical outcomes of ABI were analyzed.Results Of the 28 patients,22 were males and 6 were females with mean age of 38 (range,7--69) years.Twenty-five (89.3%) had descending thoracic aortic injury,two (7.1%) had abdominal aortic injury and one (3.6%) presented with multiple aortic injuries.Three patients (10.7%) with Grade Ⅰ,1 (3.6%) Grade Ⅱ,22 (78.6%) Grade Ⅲ,and 2 (7.1%) Grade Ⅳ injuries.Twenty-five patients underwent thoracic endovascular aortic repair and 3 were managed medically.Median time between injury and surgical intervention was (2±1) days.One (3.6%) patient developed paraplegia after thoracic endovascular aortic repair (TEVAR).One Type 2 endoleak spontaneously sealed within 1 month,and another patient died from ruptured Type 1 endoleak 3 years later.Median follow-up time was 16 (range,1-96) months.Perioperative 30-day mortality rate was 3.6%.Conclusions This study based on our modified BAI grading system indicated that Grade Ⅰ BAI can be managed conservatively.Grade Ⅱ injury requires close observation and repeated computerized tomography angiogram (CTA)within 48-72 hours.If injury appears worse on follow up imaging,surgery should be performed.Delayed repair of Grade Ⅲ BAI is acceptable if associated life threatening traumatic

  10. Endovascular repair of aortic aneurysm: Preliminary results

    Directory of Open Access Journals (Sweden)

    Davidović Lazar

    2009-01-01

    Full Text Available Introduction. Endovascular aneurysm repair (EVAR has been introduced into clinical practice at the beginning of the 90's of the last century. Because of economic, political and social problems during the last 25 years, the introduction of this procedure in Serbia was not possible. Objective. The aim of this study was to present preliminary experiences and results of the Clinic for Vascular Surgery of the Serbian Clinical Centre in Belgrade in endovascular treatment of thoracic and abdominal aortic aneurysms. Methods. The procedure was performed in 33 patients (3 female and 30 male, aged from 42 to 83 years. Ten patients had a descending thoracic aorta aneurysm (three atherosclerotic, four traumatic - three chronic and one acute as a part of polytrauma, one dissected, two penetrated atherosclerotic ulcers, while 23 patients had the abdominal aortic aneurysm, one ruptured and two isolated iliac artery aneurysms. The indications for EVAR were isthmic aneurismal localisation, aged over 80 years and associated comorbidity (cardiac, pulmonary and cerebrovasular diseases, previous thoracotomy or multiple laparotomies associated with abdominal infection, idiopatic thrombocitopaenia. All of these patients had three or more risk factors. The diagnosis was established using duplex ultrasonography, angiography and MSCT. In the case of thoracic aneurysm, a Medtronic-Valiant® endovascular stent graft was implanted, while for the abdominal aortic aneurysm Medtronic-Talent® endovascular stent grafts with delivery systems were used. In three patients, following EVAR a surgical repair of the femoral artery aneurysm was performed, and in another three patients femoro-femoral cross over bypass followed implantation of aortouniiliac stent graft. Results. During procedure and follow-up period (mean 1.6 years, there were: one death, one conversion, one endoleak type 1, six patients with endoleak type 2 that disappeared during the follow-up period, one early graft

  11. Decreased expression of fibulin-4 in aortic wall of aortic dissection.

    Science.gov (United States)

    Huawei, P; Qian, C; Chuan, T; Lei, L; Laing, W; Wenlong, X; Wenzhi, L

    2014-02-01

    In this research, we will examine the expression of Fibulin-4 in aortic wall to find out its role in aortic dissection development. The samples of aortic wall were obtained from 10 patients operated for acute ascending aortic dissection and five patients for chronic ascending aortic dissection. Another 15 pieces of samples from patients who had coronary artery bypass were as controls. The aortic samples were stained with aldehyde magenta dyeing to evaluate the arrangement of elastic fibers. The Fibulin-4 protein and mRNA expression were both determined by Western blot and realtime quantitative polymerase chain reaction. Compared with the control group, both in acute and chronic ascending aortic dissection, elastic fiber fragments increased and the expression of fibulin-4 protein significantly decreased (P= 0.045 < 0.05). The level of fibulin-4 mRNA decreased in acute ascending aortic dissection (P= 0.034 < 0.05), while it increased in chronic ascending aortic dissection (P=0.004 < 0.05). The increased amounts of elastic fiber fragments were negatively correlated with the expression of fibulin-4 mRNA in acute ascending aortic dissection. In conclusion, in aortic wall of ascending aortic dissection, the expression of fibulin-4 protein decreased and the expression of fibulin-4 mRNA was abnormal. Fibulin-4 may play an important role in the pathogenesis of aortic dissection.

  12. Management of Concomitant Cancer and Abdominal Aortic Aneurysm

    Directory of Open Access Journals (Sweden)

    Abdullah Jibawi

    2011-01-01

    Full Text Available Background. The coexistence of neoplasm and abdominal aortic aneurysm (AAA presents a real management challenge. This paper reviews the literature on the prevalence, diagnosis, and management dilemmas of concurrent visceral malignancy and abdominal aortic aneurysm. Method. The MEDLINE and HIGHWIRE databases (1966-present were searched. Papers detailing relevant data were assessed for quality and validity. All case series, review articles, and references of such articles were searched for additional relevant papers. Results. Current challenges in decision making, the effect of major body-cavity surgery on an untreated aneurysm, the effects of major vascular surgery on the treatment of malignancy, the use of EVAR (endovascular aortic aneurysm repair as a fairly low-risk procedure and its role in the management of malignancy, and the effect of other challenging issues such as the use of adjuvant therapy, and patients informed decision-making were reviewed and discussed. Conclusion. In synchronous malignancy and abdominal aortic aneurysm, the most life-threatening lesion should be addressed first. Endovascular aneurysm repair where possible, followed by malignancy resection, is becoming the preferred initial treatment choice in most centres.

  13. Transcatheter aortic valve implantation versus surgical aortic valve replacement for severe aortic stenosis: a meta analysis

    Institute of Scientific and Technical Information of China (English)

    WU Yi-cheng; ZHANG Jian-feng; SHEN Wei-feng; ZHAO Qiang

    2013-01-01

    Background Transcatheter aortic valve implantation (TAVI) has emerged as the treatment choice for non-operable patients with severe symptomatic aortic stenosis (AS) and may be a good alternative to surgery for those at very high or prohibitive surgical risk.We performed a meta-analysis to evaluate the comparative benefits of TAVI versus surgical aortic valve replacement (SAVR) in patients with severe AS.Methods A comprehensive literature search of PubMed,Embase,ScienceDirect and Cochrane Central Register of Controlled trials was performed,and randomized trials as well as cohort studies with propensity score analysis were included.Results One randomized trial (n=699) and six retrospective cohort studies (n=781) were selected for meta-analysis.Mortality at 30-day and 1-year follow-up was comparable between TAVI and SAVR.Despite similar incidences of stroke,myocardial infarction,re-operation for bleeding,and renal failure requiring dialysis,TAVI was associated with a lower occurrence rate of new-onset atrial fibrillation (OR 0.51,95% CI 0.33-0.78) and shorter procedural time (mean difference -67.50 minutes,95% CI-87.20 to-47.81 minutes).Post-operative aortic regurgitation and permanent pacemaker implantation were more common in patients after TAVI than in those with SAVR (OR 5.53,95% CI 3.41-8.97; OR 1.71,95% Cl 1.02-2.84,respectively).Conclusion In patients with severe symptomatic AS,TAVI and SAVR did not differ with respect to short-and mid-term survival,but the incidence of permanent pacemaker implantation and post-procedural aortic regurgitation remain relatively high after TAVI.

  14. Aortic Dissection of Unknown Origin in a Young Patient: A Case Report

    OpenAIRE

    Majid Hajimaghsoudi; Faeze Zeinali; Mehdi Bagherabadi; Morteza Saeedi

    2016-01-01

    Aortic dissection occurs when a tear develops in the wall of the aorta, which is rare in the young population. This fatal disorder is hard to diagnose, especially in young patients. We present the case of aortic dissection in a 15-year-old boy referred to the Emergency Department of Yazd University of Medical Sciences in November 2015. The patient presented to our department with sudden acute chest pain. Emergent computed tomography (CT) scanning of the brain, chest, and abdomen reflected bil...

  15. Transesophageal echocardiographic evaluation of an intraoperative retrograde acute aortic dissection: case report

    Directory of Open Access Journals (Sweden)

    Reiter Charles G

    2006-04-01

    Full Text Available Abstract Background We report an intraoperative retrograde dissection of the aorta and its subsequent evaluation by transesophageal echocardiography (TEE. Case presentation A 78 year old woman with an ascending aortic aneurysm without dissection and coronary artery disease was brought to the operating room for aneurysm repair and coronary artery bypass grafting. After initiation of cardiopulmonary bypass through a femoral artery cannula, aortic dissection was noted and subsequently imaged by TEE. Conclusion Retrograde aortic dissection through the femoral artery is life-threatening. Intraoperative TEE can be used to diagnose this uncommon event, and should be considered after initiation of bypass.

  16. Abdominal Aortic Dissection in a Patient With Autosomal Dominant Polycystic Kidney Disease After Starting Peritoneal Dialysis

    Directory of Open Access Journals (Sweden)

    Yingying He

    2014-07-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD, one of the most common genetic disorders, is caused by mutations in the PKD1 or PKD2 gene. ADPKD primarily affects the kidneys, causing the development of multiple bilateral cysts that are characteristic of this condition. Besides renal abnormalities, other manifestations of ADPKD include hepatic, pancreatic, and splenic cysts, intracranial aneurysms, aortic aneurysms, and mitral valve prolapse. Reports of ADPKD-associated abdominal aortic dissections are not rare, but there have been no reports of an ADPKD patient developing intestinal obstruction and abdominal aortic dissection after initiating peritoneal dialysis. Herein, we present one such case.

  17. Chimney and periscope technique for emergent treatment of spontaneous aortic rupture.

    Science.gov (United States)

    Trellopoulos, George; Georgakarakos, Efstratios; Pelekas, Dimitrios; Papachristodoulou, Athanasia; Argyriou, Christos; Georgiadis, George S

    2014-07-01

    Aortic rupture comprises a potentially fatal condition necessitating emergent treatment. Endovascular sealing of the rupture site is often combined with the use of chimney- and periscope stent placement to preserve perfusion of aortic branches. We present a case of successful endovascular management of contained aortic rupture in a 78-year-old patient. The left brachial access facilitated stenting of the celiac and superior mesenteric arteries, whereas the left femoral route served stenting of the renal artery. One-month follow-up confirmed complete sealing, stent patency, and absence of endograft migration. The combined periscope and chimney technique is feasible and effective in the emergency setting. PMID:24517987

  18. Endovascular management of an acquired aortobronchial fistula following aortic bypass for coarctation.

    LENUS (Irish Health Repository)

    O'Sullivan, Katie E

    2013-09-20

    Aortobronchial fistula (ABF) in the setting of aortic coarctation repair is very rare but uniformly fatal if untreated. Endovascular stenting of the descending aorta is now the first-choice approach for ABF presenting with haemoptysis and offers a less-invasive technique with improved outcomes, compared with open repair. We report a case of late ABF occurring following bypass for aortic coarctation. Management focused on two key manoeuvres: use of a covered endovascular stent to occlude the aortic bypass thus controlling the fistula and dilatation and stenting of native coarctation.

  19. Endovascular management of an acquired aortobronchial fistula following aortic bypass for coarctation.

    Science.gov (United States)

    O'Sullivan, Katie E; Bolster, Ferdia; Lawler, Leo P; Hurley, John

    2014-01-01

    Aortobronchial fistula (ABF) in the setting of aortic coarctation repair is very rare but uniformly fatal if untreated. Endovascular stenting of the descending aorta is now the first-choice approach for ABF presenting with haemoptysis and offers a less-invasive technique with improved outcomes, compared with open repair. We report a case of late ABF occurring following bypass for aortic coarctation. Management focused on two key manoeuvres: use of a covered endovascular stent to occlude the aortic bypass thus controlling the fistula and dilatation and stenting of native coarctation.

  20. Indexing aortic valve area by body surface area increases the prevalence of severe aortic stenosis

    DEFF Research Database (Denmark)

    Jander, Nikolaus; Gohlke-Bärwolf, Christa; Bahlmann, Edda;

    2014-01-01

    To account for differences in body size in patients with aortic stenosis, aortic valve area (AVA) is divided by body surface area (BSA) to calculate indexed AVA (AVAindex). Cut-off values for severe stenosis are......To account for differences in body size in patients with aortic stenosis, aortic valve area (AVA) is divided by body surface area (BSA) to calculate indexed AVA (AVAindex). Cut-off values for severe stenosis are...

  1. Congenital aortic regurgitation in a child with a tricuspid non-stenotic aortic valve.

    OpenAIRE

    Hashimoto, R; Miyamura, H; Eguchi, S

    1984-01-01

    After follow up for seven years a 10 year old boy with congenital aortic regurgitation was found to have a tricuspid non-stenotic aortic valve at operation. The right coronary cusp was dysplastic, thickened, and contracted; the gap between its free margin and aortic wall was bridged with two fibrous bands; and the left coronary and non-coronary cusps were almost normal. The aortic valve was replaced with a prosthesis (St Jude Medical No 23), and the postoperative course was uneventful.

  2. Aortopathy in patients with a bicuspid aortic valve : determining susceptibility for aortic complications

    NARCIS (Netherlands)

    Grewal, Nimrat

    2015-01-01

    The prevalence of aortic dilation and related complications as rupture and dissection is higher in patients with a bicuspid aortic valve (BAV) as compared to patients with a tricuspid aortic valve (TAV), although not every individual carries this increased risk. It is therefore essential to identify

  3. The Efficacy of Autologous Femoropopliteal Vein Reconstruction for Primary Aortic and Aortic Graft Infection

    NARCIS (Netherlands)

    Dirven, M.; Jagt, M.F.P. van der; Barendregt, W.B.; Vliet, D. van der

    2015-01-01

    BACKGROUND: The objective of our study was to analyze the efficacy of autologous superficial femoropopliteal vein reconstruction for primary aortic or aortic graft infection. METHODS: We performed a retrospective analysis of 14 patients treated for an infected aortic prosthesis or primary infected a

  4. Transcatheter valve-in-valve implantation due to severe aortic regurgitation in a degenerated aortic homograft

    DEFF Research Database (Denmark)

    Olsen, Lene Kjaer; Engstrøm, Thomas; Søndergaard, Lars

    2009-01-01

    a successful valve-in-valve implantation of a CoreValve aortic valve prosthesis through the right subclavian artery in a case of severe aortic regurgitation within a degenerated aortic homograft. The case exemplifies the possibilities of expanding the indications for TAVI, as well as other vascular access...

  5. New-Onset Atrial Fibrillation After Surgical Aortic Valve Replacement and Transcatheter Aortic Valve Implantation

    DEFF Research Database (Denmark)

    Jørgensen, Troels Højsgaard; Thygesen, Julie Bjerre; Thyregod, Hans Gustav;

    2015-01-01

    Surgical aortic valve replacement (SAVR) and, more recently, transcatheter aortic valve implantation (TAVI) have been shown to be the only treatments that can improve the natural cause of severe aortic valve stenosis. However, after SAVR and TAVI, the incidence of new-onset atrial fibrillation...

  6. New-onset atrial fibrillation after surgical aortic valve replacement and transcatheter aortic valve implantation

    DEFF Research Database (Denmark)

    Jørgensen, Troels Højsgaard; Thygesen, Julie Bjerre; Thyregod, Hans Gustav;

    2015-01-01

    Surgical aortic valve replacement (SAVR) and, more recently, transcatheter aortic valve implantation (TAVI) have been shown to be the only treatments that can improve the natural cause of severe aortic valve stenosis. However, after SAVR and TAVI, the incidence of new-onset atrial fibrillation...

  7. MARFAN SYNDROME AND QUADRICUSPID AORTIC VALVE

    OpenAIRE

    Sürücü, Hüseyin; ÇİMEN, Sadi

    2013-01-01

    ABSTRACTWhile the major cardiovascular manifestation in Marfan syndrome is a progressive dilatation of the ascending aorta, leading to aortic aneurysm formation and eventually to fatal aortic rupture or dissection, mitral valve prolapse and calcification of the mitral valve annulus, dilatation of the main pulmonary artery may be seen. There was no knowledge about the association of Marfan syndrome and quadricuspid aortic valve. In this case report, we aimed to declare this association between...

  8. Transcatheter Aortic Valve Replacement in a Nonagenarian

    OpenAIRE

    Kneitz, Abby; Clifton, William; Kar, Biswajit; Delgado, Reynolds M.

    2013-01-01

    Approximately 30% to 40% of elderly patients with severe, symptomatic aortic valve stenosis are deemed ineligible for surgery because of high perioperative risk. We describe the use of an alternative transfemoral approach known as transcatheter aortic valve implantation in a nonagenarian patient with severe aortic stenosis. Our patient recovered successfully, and by the time of her most recent follow-up visit, 7 months after the procedure, she had regained a substantial degree of function. Th...

  9. Recessive mutations in SLC38A8 cause foveal hypoplasia and optic nerve misrouting without albinism.

    Science.gov (United States)

    Poulter, James A; Al-Araimi, Musallam; Conte, Ivan; van Genderen, Maria M; Sheridan, Eamonn; Carr, Ian M; Parry, David A; Shires, Mike; Carrella, Sabrina; Bradbury, John; Khan, Kamron; Lakeman, Phillis; Sergouniotis, Panagiotis I; Webster, Andrew R; Moore, Anthony T; Pal, Bishwanath; Mohamed, Moin D; Venkataramana, Anandula; Ramprasad, Vedam; Shetty, Rohit; Saktivel, Murugan; Kumaramanickavel, Govindasamy; Tan, Alex; Mackey, David A; Hewitt, Alex W; Banfi, Sandro; Ali, Manir; Inglehearn, Chris F; Toomes, Carmel

    2013-12-01

    Foveal hypoplasia and optic nerve misrouting are developmental defects of the visual pathway and only co-occur in connection with albinism; to date, they have only been associated with defects in the melanin-biosynthesis pathway. Here, we report that these defects can occur independently of albinism in people with recessive mutations in the putative glutamine transporter gene SLC38A8. Nine different mutations were identified in seven Asian and European families. Using morpholino-mediated ablation of Slc38a8 in medaka fish, we confirmed that pigmentation is unaffected by loss of SLC38A8. Furthermore, by undertaking an association study with SNPs at the SLC38A8 locus, we showed that common variants within this gene modestly affect foveal thickness in the general population. This study reveals a melanin-independent component underpinning the development of the visual pathway that requires a functional role for SLC38A8.

  10. Focal dermal hypoplasia due to a novel mutation in a boy with Klinefelter syndrome.

    Science.gov (United States)

    Alkindi, Said; Battin, Malcolm; Aftimos, Salim; Purvis, Diana

    2013-01-01

    A boy was born with multiple anomalies, including right hemifacial microsomia, eye abnormalities, syndactyly, right hand ectrodactyly, hypoplastic nails, omphalocele, bladder exstrophy, renal dilatation, and splayed symphysis pubis. The skin was also abnormal, with atrophic skin plaques and areas of telangiectasia along the lines of Blaschko. The karyotype was 47,XXY (Klinefelter syndrome). He was found to have a heterozygous mutation in the PORCN gene. He exhibited the classical features of focal dermal hypoplasia. Fewer than 15% of reported cases are male when it is thought to be due to postzygotic mutation and thus mosaic. This is the first reported boy to have heterozygous mutation for Goltz syndrome who survived due to the extra X chromosome. PMID:23131169

  11. Sudden death in infancy due to bicuspid aortic valve.

    Science.gov (United States)

    Karayel, Ferah; Ozaslan, Abdi; Turan, Arzu Akcay; Pakis, Isil; Ketenci, Cetin; Eroglu, Ayse Guler

    2006-09-01

    Symptoms of bicuspid aortic valve usually occur in the age group of 50-70 years, but rarely, it can also lead to sudden unexpected death in infancy and early childhood. The autopsy of a 2-month-old baby boy, found dead in his cot, revealed the heart weight as 25 g, and the macroscopic examination showed the circumference of the aortic valve consisting of two leaflets as 8 mm. The thickness of the left ventricle, right ventricle, and septum was measured as 8, 7, and 10 mm, respectively. Microscopically, the heart revealed hypertrophic changes of myocytes. Subendocardial areas displayed necrosis of myocytes, and severe and diffuse ischemic changes characterized by loss of myofibers and vacuolization. Interstitial pneumonia was identified in the lungs. Death occurred as a result of a congenital bicuspid aortic valve obstructing the left ventricular outflow tract complicated by lung infection. As there are only a few reported cases in infancy, and congenital bicuspid aortic valve can lead to sudden unexpected death, this case is presented to the forensic community.

  12. Medical image of the week: acute aortic dissection

    Directory of Open Access Journals (Sweden)

    Desai H

    2015-06-01

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

  13. Simulation study and function analysis of the dynamic aortic valve

    Institute of Scientific and Technical Information of China (English)

    XIA Dongdong; BAI Jing

    2006-01-01

    The dynamic aortic valve (DAV) is a new left ventricular assist device, a micro-axial blood pump implemented at the position of the aortic valve, pumping blood from the left ventricle into the aortic artery. The present dynamic aortic valve operates at 7 different rotation speeds, ranging from 3000 r/min (speed 1) to 9000 r/min (speed 7). Because in vivo experiments need a lot of live animals and take a long period of time, modeling and simulation have been widely used to simulate and analyze hydra-dynamic property of the DAV and its assisting effects. With the measurements from the mock circulatory loop, a mathematic model of the DAV is established and embedded into the previously developed canine circulatory system. Using this model, the effect of the DAV on the failing heart at each rotation speed level is investigated. The vital cardiac variables are computed and compared with in vivo experimental results, which are in good agreement with an acceptable difference mostly 15 %. The establishment of the DAV model and its simulation are useful for further improvement of the DAV device.

  14. Wall shear stress indicators in abnormal aortic geometries

    Science.gov (United States)

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

    2015-11-01

    Cardiovascular disease, such as atherosclerosis, occurs at specific locations in the arterial tree. Characterizing flow and forces at these locations is crucial to understanding the genesis of disease. Measures such as time average wall shear stress, oscillatory shear index, relative residence time and temporal wall shear stress gradients have been shown to identify plaque prone regions. The present paper examines these indices in three aortic geometries obtained from patients whose aortas are deformed due to a genetic pathology and compared to one normal geometry. This patient group is known to be prone to aortic dissection and our study aims to identify early indicators that will enable timely intervention. Data obtained from cardiac magnetic resonance imaging is used to reconstruct the aortic arch. The local unsteady flow characteristics are calculated, fully resolving the flow field throughout the entire cardiac cycle. The Quemada model is applied to account for the non-Newtonian properties of blood, an empirical model valid for different red blood cell loading. The impact of the deformed aortic geometries is analyzed to identify flow patterns that could lead to arterial disease at certain locations.

  15. Genetic analysis of NR0B1 in congenital adrenal hypoplasia patients: identification of a rare regulatory variant resulting in congenital adrenal hypoplasia and hypogonadal hypogonadism without testicular carcinoma in situ.

    Science.gov (United States)

    Walker, A P; Fowkes, R C; Saleh, F; Kim, S-H; Wilkinson, P; Cabrera-Sharp, V; Talmud, P J; Humphries, S E; Looijenga, L H J; Bouloux, P M G

    2012-01-01

    There have been few testicular histology reports of adult patients with congenital adrenal hypoplasia/hypogonadal hypogonadism (AHC/HH), but Leydig cell hyperplasia has been observed, an indicator of the possibility of malignant transformation. We aimed to define the basis of AHC/HH in 4 pedigrees of different ethnic backgrounds. One patient was elected to have testicular biopsy which was examined for evidence of carcinoma in situ (CIS). NR0B1 mutation analysis was performed by sequence analysis. NR0B1 expression was investigated by RT-PCR. Testicular biopsy sections were stained with HE or immunostained for OCT3/4, an established marker of CIS. We identified NR0B1 variants in the 4 AHC pedigrees: pedigree 1 (United Arab Emirates), c.1130A>G predicting p.(Glu377Gly); pedigree 2 (English Caucasian), c.327C>A predicting p.(Cys109*); pedigree 3 (Oman), a 6-bp deletion of a direct repeat, c.857_862delTGGTGC predicting p.(Leu286_Val287del); pedigree 4 (English Caucasian), c.1168+1G>A, a regulatory variant within the NR0B1 splice donor site. This last male patient, aged 30 years, presented with evidence of HH but incomplete gonadotrophin deficiency, following an earlier diagnosis of Addison's disease at 3 years. Hormonal therapy induced virilisation. Testicular biopsy was performed. The c.1168+1G>A variant abrogated normal splicing of testicular mRNA. Histological examination showed poorly organised testicular architecture and absence of spermatozoa. Morphological analyses and the absence of immunohistochemical staining for OCT3/4 excluded the presence of malignant germ cell cancer and its precursor lesion, CIS. These studies add to the knowledge of the types and ethnic diversity of NR0B1 mutations and their associated phenotypes, and provide insight into the assessment and interpretation of testicular histology in AHC and HH. PMID:23018754

  16. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

    Energy Technology Data Exchange (ETDEWEB)

    Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net; Dagan, Tamir [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Atar, Eli; Schwartz, Michael [Schneider Children' s Medical Center Israel, Section of Radiology (Israel); Kachko, Ludmila [Schneider Children' s Medical Center Israel, Section of Anesthesiology (Israel); Superina, Riccardo; Amir, Gabriel [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Shapiro, Rivka [Schneider Children' s Medical Center Israel, Section of Gastroenterology (Israel); Birk, Einat [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel)

    2013-12-15

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by {approx}50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 {+-} 11.3 to 10.8 {+-} 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 {+-} 0.5 to 4.0 {+-} 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 {+-} 53.6 to 65.7 {+-} 9.6 {mu}mol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

  17. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

    International Nuclear Information System (INIS)

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ∼50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5–13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2–8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia

  18. Initial Surgical Experience with Aortic Valve Repair: Clinical and Echocardiographic Results

    Science.gov (United States)

    da Costa, Francisco Diniz Affonso; Colatusso, Daniele de Fátima Fornazari; da Costa, Ana Claudia Brenner Affonso; Balbi Filho, Eduardo Mendel; Cavicchioli, Vinicius Nesi; Lopes, Sergio Augusto Veiga; Ferreira, Andrea Dumsch de Aragon; Collatusso, Claudinei

    2016-01-01

    Introduction Due to late complications associated with the use of conventional prosthetic heart valves, several centers have advocated aortic valve repair and/or valve sparing aortic root replacement for patients with aortic valve insufficiency, in order to enhance late survival and minimize adverse postoperative events. Methods From March/2012 thru March 2015, 37 patients consecutively underwent conservative operations of the aortic valve and/or aortic root. Mean age was 48±16 years and 81% were males. The aortic valve was bicuspid in 54% and tricuspid in the remaining. All were operated with the aid of intraoperative transesophageal echocardiography. Surgical techniques consisted of replacing the aortic root with a Dacron graft whenever it was dilated or aneurysmatic, using either the remodeling or the reimplantation technique, besides correcting leaflet prolapse when present. Patients were sequentially evaluated with clinical and echocardiographic studies and mean follow-up time was 16±5 months. Results Thirty-day mortality was 2.7%. In addition there were two late deaths, with late survival being 85% (CI 95% - 68%-95%) at two years. Two patients were reoperated due to primary structural valve failure. Freedom from reoperation or from primary structural valve failure was 90% (CI 95% - 66%-97%) and 91% (CI 95% - 69%-97%) at 2 years, respectively. During clinical follow-up up to 3 years, there were no cases of thromboembolism, hemorrhage or endocarditis. Conclusions Although this represents an initial series, these data demonstrates that aortic valve repair and/or valve sparing aortic root surgery can be performed with satisfactory immediate and short-term results. PMID:27556321

  19. Contemporary Management of Type B Aortic Dissection in the Endovascular Era.

    Science.gov (United States)

    Bannazadeh, Mohsen; Tadros, Rami O; McKinsey, James; Chander, Rajiv; Marin, Michael L; Faries, Peter L

    2016-04-01

    Aortic dissection (AD) is one of the most common catastrophic pathologies affecting the aorta. Anatomic classification is based on the origin of entry tear and its extension. Type A dissections originate in the ascending aorta, whereas the entry tear in Type B dissections starts distal to the left subclavian artery. The patients with aortic dissection who manifest complications such as rupture, malperfusion, aneurysmal degeneration, and intractable pain are classified as complicated AD. Risk factors for developing aortic dissection include age, male gender, and aortic wall structural abnormalities. The most common presenting symptom of acute aortic dissection is pain. Malperfusion occurs as a result of end-organ ischemia due to involvement of aortic branches from the dissecting process. This can happen in various locations causing mesenteric ischemia (mesenteric vessels), stroke (aortic arch vessels), renal failure (renal arteries), spinal ischemia, and limb ischemia (iliac or subclavian arteries). Aneurysmal degeneration is the most common complication of patients with chronic Type B dissection who are managed with medical therapy. Management of Type B aortic dissection (TBAD) remains controversial. Many groups recommend conservative therapy for newly diagnosed TBAD and reserve surgical management for patients who develop complications such as rupture, malperfusion, aneurysmal dilatation, and refractory pain. The mainstay of medical therapy includes antihypertensive medication to reduced ΔP/ ΔT by lowering blood pressure and heart rate. With the continued success of thoracic endovascular aortic repair (TEVAR), this procedure has been extended to treat TBAD in selected patients. The outcomes of TEVAR are promising, with early mortality rates from 10% to 20%. With promising results from these series, some groups recommend early TEVAR in uncomplicated TBAD to prevent future adverse events. The goals of endovascular treatment of TBAD are to cover the entry tear

  20. Abdominal Aortic Surgery: Anesthetic Implications

    OpenAIRE

    Cunningham, Anthony J.

    1991-01-01

    The objectives of the review are to highlight the clinical characteristics of the patient population; to assess multivariate risk factor analysis and the invasive/non-invasive techniques available for risk factor identification and management in this high-risk surgical population; to assess the major hemodynamic, metabolic, and regional blood flow changes associated with aortic cross-clamping/unclamping procedures and techniques for their modification or attenuation; and to assess the influen...

  1. SURGICAL MANAGEMENT OF A RARE CASE OF MARFAN SYNDROME - THE RUPTURE OF AN ABDOMINAL AORTIC ANEURYSM

    Directory of Open Access Journals (Sweden)

    L. Stoica

    2005-10-01

    Full Text Available We present a rare manifestation of the Marfan syndrome. A 33 years old woman with a 10 cm diameter juxta-renal aortic aneurysm ruptured in the retro peritoneum arrived in hemorrhagic shock. Surgery was performed in emergency by thoraco-phreno-laparatomy, the aneurysm was resected and a 20 mm Dacron tube was sutured between the origin of the renal arteries and the aortic bifurcation. Rapid thoracic aortic cross-clamping to stop the hemorrhage and good aneurismal exposure by the division of the left renal vein was the key of this successful management. The patient recovered without any complication. Rupture of the abdominal aortic aneurysm is a life threatening complication which requires a rapid diagnosis and an emergency treatment by open surgery or by endovascular stent grafting. We present the surgical strategy in our case

  2. Repair of recurrent pseudoaneurysm of the mitral-aortic intervalvular fibrosa: Role of transesophageal echocardiography

    Directory of Open Access Journals (Sweden)

    Shreedhar S Joshi

    2014-01-01

    Full Text Available Pseudoaneurysm of mitral-aortic intervalvular fibrosa (P-MAIVF is a rare cardiac surgical condition. P-MAIVF commonly occurs as a complication of aortic and mitral valve replacement surgeries. The surgical trauma during replacement of the valves weakens the avascular mitral and aortic intervalvular area. We present a case of P-MAIVF recurrence 5 years after a primary repair. Congestive cardiac failure was the presenting feature with mitral and aortic regurgitation. In view of the recurrence, the surgical team planned for a double valve replacement. The sewing rings of the two prosthetic-valves were interposed to close the mouth of the pseudoaneurysm and to provide mechanical reinforcement of the MAIVF. Intra-operative transesophageal echocardiography (TEE helped in delineating the anatomy, extent of the lesion, rupture of one of the pseudoaneurysm into left atrium and severity of the valvular regurgitation. Post-procedure TEE confirmed complete obliteration of the pseudoaneurysm and prosthetic valve function.

  3. Lower respiratory tract infection and rapid expansion of an abdominal aortic aneurysm: a case report

    Directory of Open Access Journals (Sweden)

    Puppala Sapna

    2010-10-01

    Full Text Available Abstract Introduction The rate of abdominal aortic aneurysm expansion is related to multiple factors. There is some evidence that inflammation can accelerate aneurysm expansion. However, the association between pulmonary sepsis and rapid abdominal aortic aneurysm expansion is rarely reported. Case presentation Here we present a case of a rapidly expanding abdominal aortic aneurysm in a 68-year-old Caucasian man with a concomitant lower respiratory tract infection and systemic sepsis requiring intensive monitoring and urgent endovascular intervention. Our patient had an uncomplicated post-operative recovery and a follow-up computed tomography scan at one month demonstrated no evidence of an endoleak. Conclusion This case highlights the potential association between pulmonary sepsis and rapid abdominal aortic aneurysm expansion. In such cases, a policy of frequent monitoring should be adopted to identify those patients requiring definitive management.

  4. 9.1 cm abdominal aortic aneurysm in a 69-year-old male patient

    Institute of Scientific and Technical Information of China (English)

    Celine; Saade; Bhavi; Pandya; Muhammad; Raza; Mustafain; Meghani; Deepak; Asti; Foad; Ghavami

    2015-01-01

    We are presenting a case of one of the largest unruptured abdominal aortic aneurysm ever reported. Presented here is a rare case of a 69-year-old active smoker male with history of hypertension and incidental diagnosis of abdominal aortic aneurysm of 6.2 cm in 2003, who refused surgical intervention at the time of diagnosis with continued smoking habit and was managed medically. Patient was subsequently admitted in 2012 to the hospital due to unresponsiveness secondary to hypoglycemia along with diagnosis of massive symptomatic pulmonary embolism and nonST elevation myocardial infarction. With the further inpatient workup along with known history of abdominal aortic aneurysm, subsequent computed tomography scan of abdomen pelvis revealed increased in size of infrarenal abdominal aortic aneurysm to 9.1 cm of without any signs of rupture. Patient was unable to undergo any surgical intervention this time because of his medical instability and was eventually passed away under hospice care.

  5. Thoracic Endovascular Aortic Repair in a Patient with Mobile Aortic Thrombosis

    Directory of Open Access Journals (Sweden)

    Graham M. Lohrmann

    2014-01-01

    Full Text Available A 58-year-old female presented with acute arterial insufficiency to her left leg. Following cardiovascular evaluation using multimodality imaging, it was discovered that she had mobile thoracic thrombi overlying a normal descending thoracic aorta which had also caused a splenic infarction. This patient was treated with unfractionated heparin for three days and underwent subsequent thoracic endovascular aortic repair (TEVAR uneventfully with no subsequent complications at one-year followup. This case highlights the diagnostic and therapeutic challenges in treating patients with this uncommon challenging clinical scenario.

  6. A novel mutation in TTC19 associated with isolated complex III deficiency, cerebellar hypoplasia, and bilateral basal ganglia lesions.

    Science.gov (United States)

    Melchionda, Laura; Damseh, Nadirah S; Abu Libdeh, Bassam Y; Nasca, Alessia; Elpeleg, Orly; Zanolini, Alice; Ghezzi, Daniele

    2014-01-01

    Isolated complex III (cIII) deficiency is a rare biochemical finding in mitochondrial disorders, mainly associated with mutations in mitochondrial DNA MTCYB gene, encoding cytochrome b, or in assembly factor genes (BCS1L, TTC19, UQCC2, and LYRM7), whereas mutations in nuclear genes encoding cIII structural subunits are extremely infrequent. We report here a patient, a 9 year old female born from first cousin related parents, with normal development till 18 months when she showed unsteady gait with frequent falling down, cognitive, and speech worsening. Her course deteriorated progressively. Brain MRI showed cerebellar vermis hypoplasia and bilateral lentiform nucleus high signal lesions. Now she is bed ridden with tetraparesis and severely impaired cognitive and language functions. Biochemical analysis revealed isolated cIII deficiency in muscle, and impaired respiration in fibroblasts. We identified a novel homozygous rearrangement in TTC19 (c.213_229dup), resulting in frameshift with creation of a premature termination codon (p.Gln77Argfs*30). Western blot analysis demonstrated the absence of TTC19 protein in patient's fibroblasts, while Blue-Native Gel Electrophoresis analysis revealed the presence of cIII-specific assembly intermediates. Mutations in TTC19 have been rarely associated with mitochondrial disease to date, being described in about ten patients with heterogeneous clinical presentations, ranging from early onset encephalomyopathy to adult forms with cerebellar ataxia. Contrariwise, the biochemical defect was a common hallmark in TTC19 mutant patients, confirming the importance of TTC19 in cIII assembly/stability. Therefore, we suggest extending the TTC19 mutational screening to all patients with cIII deficiency, independently from their phenotypes. PMID:25452764

  7. 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.

  8. An up-to-date overview of the most recent transcatheter implantable aortic valve prostheses.

    Science.gov (United States)

    Wiegerinck, Esther M A; Van Kesteren, Floortje; Van Mourik, Martijn S; Vis, Marije M; Baan, Jan

    2016-01-01

    Over the past decade transcatheter aortic valve implantation (TAVI) has evolved towards the routine therapy for high-risk patients with severe aortic valve stenosis. Technical refinements in TAVI are rapidly evolving with a simultaneous expansion of the number of available devices. This review will present an overview of the current status of development of TAVI-prostheses; describes the technical features and applicability of each device and the clinical data available.

  9. Ruptured Juxtarenal Abdominal Aortic Aneurysm Treated With a Fenestrated EVAR Graft Intended for a Different Patient.

    Science.gov (United States)

    Al-Jundi, Wissam; Elboushi, Amro; Lees, Tim; Williams, Robin

    2016-08-01

    Treatment of juxtarenal abdominal aortic aneurysms (AAAs) remains challenging. A 79-year-old male who had infrarenal endovascular repair of abdominal aortic aneurysm (EVAR) 13 years previously presented with leaking juxtarenal AAA. Emergency fenestrated EVAR (FEVAR) was performed utilizing a stent graft designed and built for a different patient. Despite the need to embolize the celiac artery prior to covering it with the stent graft in order to achieve adequate proximal seal, the patient had uneventful recovery. PMID:27334480

  10. The role of transesophageal echocardiography in aortic surgery.

    Science.gov (United States)

    Nowak-Machen, Martina

    2016-09-01

    Aortic disease, when left untreated, is still associated with major morbidity and mortality. Aortic dissection and aortic aneurysm are the main reasons for performing aortic surgery procedures in the adult. Imaging techniques such as computed tomography and magnetic resonance imaging play a key role in the preoperative evaluation. Transesophageal echocardiography (TEE) has become a safe and invaluable perioperative imaging tool for aortic disease over the past decade with high sensitivity and specificity. TEE can increase patient safety and improve overall patient outcome in aortic surgery. Especially during endovascular aortic repair, TEE is more sensitive than other imaging modalities in diagnosing complications such as graft endoleaks. Newer echocardiographic techniques such as three-dimensional (3D) TEE and contrast-enhanced TEE are emerging and seem to have a valuable role especially in aortic dissection repair and endovascular aortic stent procedures. In the absence of contraindications, TEE should generally be performed during aortic surgery and endovascular aortic procedures. PMID:27650342

  11. The focal adhesion: a regulated component of aortic stiffness.

    Directory of Open Access Journals (Sweden)

    Robert J Saphirstein

    Full Text Available Increased aortic stiffness is an acknowledged predictor and cause of cardiovascular disease. The sources and mechanisms of vascular stiffness are not well understood, although the extracellular matrix (ECM has been assumed to be a major component. We tested here the hypothesis that the focal adhesions (FAs connecting the cortical cytoskeleton of vascular smooth muscle cells (VSMCs to the matrix in the aortic wall are a component of aortic stiffness and that this component is dynamically regulated. First, we examined a model system in which magnetic tweezers could be used to monitor cellular cortical stiffness, serum-starved A7r5 aortic smooth muscle cells. Lysophosphatidic acid (LPA, an activator of myosin that increases cell contractility, increased cortical stiffness. A small molecule inhibitor of Src-dependent FA recycling, PP2, was found to significantly inhibit LPA-induced increases in cortical stiffness, as well as tension-induced increases in FA size. To directly test the applicability of these results to force and stiffness development at the level of vascular tissue, we monitored mouse aorta ring stiffness with small sinusoidal length oscillations during agonist-induced contraction. The alpha-agonist phenylephrine, which also increases myosin activation and contractility, increased tissue stress and stiffness in a PP2- and FAK inhibitor 14-attenuated manner. Subsequent phosphotyrosine screening and follow-up with phosphosite-specific antibodies confirmed that the effects of PP2 and FAK inhibitor 14 in vascular tissue involve FA proteins, including FAK, CAS, and paxillin. Thus, in the present study we identify, for the first time, the FA of the VSMC, in particular the FAK-Src signaling complex, as a significant subcellular regulator of aortic stiffness and stress.

  12. Regional aortic distensibility and its relationship with age and aortic stenosis: a computed tomography study.

    Science.gov (United States)

    Wong, Dennis T L; Narayan, Om; Leong, Darryl P; Bertaso, Angela G; Maia, Murilo G; Ko, Brian S H; Baillie, Timothy; Seneviratne, Sujith K; Worthley, Matthew I; Meredith, Ian T; Cameron, James D

    2015-06-01

    Aortic distensibility (AD) decreases with age and increased aortic stiffness is independently associated with adverse cardiovascular outcomes. The association of severe aortic stenosis (AS) with AD in different aortic regions has not been evaluated. Elderly subjects with severe AS and a cohort of patients without AS of similar age were studied. Proximal aortic cross-sectional-area changes during the cardiac cycle were determined using retrospective-ECG-gating on 128-detector row computed-tomography. Using oscillometric-brachial-blood-pressure measurements, the AD at the ascending-aorta (AA), proximal-descending-aorta (PDA) and distal-descending-aorta (DDA) was determined. Linear mixed effects modelling was used to determine the association of age and aortic stenosis on regional AD. 102 patients were evaluated: 36 AS patients (70-85 years), 24 AS patients (>85 years) and 42 patients without AS (9 patients DDA (1.1 ± 1.2 vs. 1.2 ± 0.8, P = 0.97). In patients without AS, AD decreased with age in all aortic regions (P < 0.001). The AA in patients <50 years were the most distensible compared to other aortic regions. There is regional variation in aortic distensibility with aging. Patients with aortic stenosis demonstrated regional differences in aortic distensibility with lower distensibility demonstrated in the proximal ascending aorta compared to an age-matched cohort. PMID:25855464

  13. Aortic Dissection and Thrombosis Diagnosed by Emergency Ultrasound in a Patient with Leg Pain and Paralysis

    Directory of Open Access Journals (Sweden)

    Ann H. Tsung

    2013-01-01

    Full Text Available The authors present a case of aortic dissection and abdominal aortic aneurysm thrombosis in a 78-year-old male who presented to the emergency department (ED complaining of lower extremity and paralysis for the past 1.5 hours. The initial vital signs in the ED were as follows: blood pressure (BP 132/88 mmHg, heart rate (HR 96, respiratory rate (RR 14, and an oxygen saturation of 94% at room air. Physical exam was notable for pale and cold left leg. The ED physician was unable to palpate or detect a Doppler signal in the left femoral artery. Bedside ultrasound was performed which showed non-pulsatile left femoral artery and limited flow on color Doppler. Abdominal aortic aneurysm screening ultrasound was performed showing a 4.99 cm infrarenal abdominal aortic aneurysm and an intra-aortic thrombus with an intimal flap. Vascular surgery was promptly contacted and the patient underwent emergent aorto-bi-femoral bypass, bilateral four compartment fasciotomy, right common femoral artery endarterectomy with profundoplasty, and subsequent left leg amputation. Emergency physicians should utilize bedside ultrasound in patients who present with risk factors or threatening signs and symptoms that may suggest aortic dissection or aneurysm. Bedside ultrasound decreases time to definitive treatment and the mortality of the patients.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2005-03-01

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

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

    Science.gov (United States)

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

    1995-01-01

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

  16. Mycotic Saccular Abdominal Aortic Aneurysm in an Infant after Cardiac Catheterization: A Case Report.

    Science.gov (United States)

    Benrashid, Ehsan; McCoy, Christopher C; Rice, Henry E; Shortell, Cynthia K; Cox, Mitchell W

    2015-10-01

    Abdominal aortic aneurysms (AAAs) are a rare entity in the pediatric population. Children with mycotic (infectious) AAA in particular are at risk of life-threatening rupture due to their rapid expansion coupled with aortic wall thinning and deterioration. Here, we present the case of a 10-month-old infant with prior 2-staged repair for hypoplastic left heart syndrome that was incidentally discovered to have a mycotic AAA on abdominal ultrasound (US) for evaluation of renovascular hypertension. Before the time of evaluation with US, the infant had developed methicillin-resistant Staphylococcus aureus bacteremia 3 days after cardiac catheterization with percutaneous thoracic aortic balloon angioplasty. She had normal aortic contours on contrasted computed tomography scan of the abdomen approximately 2 weeks before the aforementioned US evaluation. This infant subsequently underwent open aneurysmorrhaphy with cryopreserved vein patch angioplasty with resolution of her aneurysmal segment.

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

    Science.gov (United States)

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

    2004-10-01

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

  18. Infective endocarditis in bicuspid aortic valve: atrioventricular block as sign of perivalvular abscess.

    Science.gov (United States)

    Bacchion, Francesco; Cukon, Sonja; Rizzoli, Giulio; Gerosa, Gino; Daliento, Luciano; Thiene, Gaetano; Basso, Cristina

    2007-01-01

    A 46-year-old man presenting with fever, peripheral edema, and chest pain was admitted to the emergency department. Electrocardiogram showed sinus tachycardia and first-degree atrioventricular block. Transesophageal echocardiogram showed infective endocarditis in bicuspid aortic valve, complicated with severe aortic regurgitation, ring abscess, and sinus-of-Valsalva aneurysm extending to mitroaortic fibrous continuity. The patient, who was unaware of his bicuspid aortic valve condition, reported having undergone an orthodontic procedure complicated with dental abscess 1 month prior, which was treated with combined clavulanate-amoxicillin antibiotic therapy. Blood cultures were positive for Bacteroides fragilis resistant to metronidazole. Intravenous antibiotic therapy was undertaken, with rapid resolution of fever. He eventually underwent successful aortic homograft implantation and mitral valve repair with residual first-degree atrioventricular block. PMID:17637435

  19. Thoracic endovascular aortic repair for complicated chronic type B aortic dissection in a patient on hemodialysis with recurrent ischemic colitis.

    Science.gov (United States)

    Miyazaki, Yuko; Furuyama, Tadashi; Matsubara, Yutaka; Yoshiya, Keiji; Yoshiga, Ryosuke; Inoue, Kentaro; Matsuda, Daisuke; Aoyagi, Yukihiko; Kato, Masaaki; Matsumoto, Takuya; Maehara, Yoshihiko

    2016-12-01

    We present a successful case of thoracic endovascular aortic repair (TEVAR) for chronic Stanford type B aortic dissection (B-AD) with recurrent ischemic colitis. The patient was a 56-year-old woman with abdominal pain as the main complaint who had two operations previously: the total arch replacement 8 years ago and the Bentall 7 years ago for acute Stanford type A aortic dissection. Her abdominal pain worsened as her blood pressure became low during her hemodialysis treatment. An enhanced computed tomography scan was performed on the patient and showed chronic B-AD that occurred from the distal anastomotic part of the total arch graft to the bilateral common iliac arteries. The celiac artery and superior mesenteric artery (SMA) arose from the true lumen, and these were compressed by the expanded false lumen. Her complicated chronic B-AD was treated with the Zenith Dissection Endovascular System, and its procedure was performed as her proximal entry tear was covered by a proximal tapered Zenith TX2 stent graft, supplemented by a noncovered aortic stent extending across both renal arteries, the SMA, and the celiac artery. Seven days after this operation, enhanced computed tomography showed that the patient's true lumen was expanded and her blood flow to the true lumen and SMA was improved. On the other hand, her false lumen tended to be thrombosed. Consequently, she was discharged 10 days after the operation without any postoperative complications as she had no abdominal complaints even though she underwent hemodialysis three times per week after the operation. We believe that TEVAR supplemented by a noncovered aortic stent is an effective treatment, even for highly chronic B-AD in dialysis patients. PMID:27090121

  20. Graft infections after surgical aortic reconstructions

    NARCIS (Netherlands)

    Berger, P.

    2015-01-01

    Prosthetic vascular grafts are frequently used to reconstruct (part) of the aorta. Every surgical procedure caries a certain risk for infection and when a prosthetic aortic graft is implanted, this may lead to an aortic graft infection (AGI). Endovascular techniques have gradually replaced open surg

  1. Thoraco-abdominal aortic aneurysm branched repair

    NARCIS (Netherlands)

    Verhoeven, E. L. G.; Tielliu, I. F. J.; Ferreira, M.; Zipfel, B.; Adam, D. J.

    2010-01-01

    Open thoraco-abdominal aortic aneurysm repair is a demanding procedure with high impact on the patient and the operating team. Results from expert centres show mortality rates between 3-21%, with extensive morbidity including renal failure and paraplegia. Endovascular repair of abdominal aortic aneu

  2. Magnetic Resonance Imaging of Thoracic Aortic Dissections

    OpenAIRE

    Sax, Steven L.

    1990-01-01

    Magnetic resonance imaging is an excellent noninvasive method for evaluating thoracic aortic dissections. A variety of magnetic resonance scans of aortic dissections are shown, documenting the ability of magnetic resonance to image the true lumen, the false channel, and the intimal septum. Detail is provided on magnetic resonance imaging techniques and findings. (Texas Heart Institute Journal 1990;17:262-70)

  3. Transcatheter Aortic Valve Replacement in Europe

    DEFF Research Database (Denmark)

    Mylotte, Darren; Osnabrugge, Ruben L J; Windecker, Stephan;

    2013-01-01

    The authors sought to examine the adoption of transcatheter aortic valve replacement (TAVR) in Western Europe and investigate factors that may influence the heterogeneous use of this therapy.......The authors sought to examine the adoption of transcatheter aortic valve replacement (TAVR) in Western Europe and investigate factors that may influence the heterogeneous use of this therapy....

  4. Global Strain in Severe Aortic Valve Stenosis

    DEFF Research Database (Denmark)

    Dahl, Jordi S; Videbæk, Lars; Poulsen, Mikael K;

    2012-01-01

    BACKGROUND: -Global longitudinal systolic strain (GLS) is often reduced in aortic stenosis despite normal ejection fraction. The importance of reduced preoperative GLS on long-term outcome after aortic valve replacement (AVR) is unknown. METHODS AND RESULTS: -A total of 125 patients with severe...

  5. Stenting for infantile adult aortic coarctation with successful conception of zygomatic twins at 4 years' post-intervention.

    LENUS (Irish Health Repository)

    Waters, Peadar S

    2013-01-01

    Aortic coarctation is a congenital defect which rarely presents in adulthood but results in significant morbidity and mortality. Endovascular techniques present novel therapeutic options for managing this anomaly with comparable results to traditional open surgical repair.

  6. Acute aortic dissection in pregnant women.

    Science.gov (United States)

    Yang, Zhaohua; Yang, Shouguo; Wang, Fangshun; Wang, Chunsheng

    2016-05-01

    Acute aortic dissection occurring during pregnancy represents a lethal risk to both the mother and fetus. Management of parturient with acute aortic dissection is complex. We report our experience of two pregnancies with type A acute aortic dissection. One patient is a 31-year-old pregnant woman (33rd gestational week) with a bicuspid aortic valve and the other is a 32-year-old pregnant woman (30th gestational week) with the Marfan syndrome. In both cases, a combined emergency operation consisting of Cesarean section, total hysterectomy prior to corrective surgery for aortic dissection was successfully performed within a relatively short period of time after the onset. Both patients' postoperative recovery was uneventful, and we achieved a favorable maternal and fetal outcome. PMID:25085319

  7. Intraoperative tracking of aortic valve plane

    Science.gov (United States)

    Nguyen, Duc Long Hung; Garreau, Mireille; Auffret, Vincent; Le Breton, Hervé; Verhoye, Jean-Philippe; Haigron, Pascal

    2013-01-01

    The main objective of this work is to track the aortic valve plane in intra-operative fluoroscopic images in order to optimize and secure Transcatheter Aortic Valve Implantation (TAVI) procedure. This paper is focused on the issue of aortic valve calcifications tracking in fluoroscopic images. We propose a new method based on the Tracking-Learning-Detection approach, applied to the aortic valve calcifications in order to determine the position of the aortic valve plane in intra-operative TAVI images. This main contribution concerns the improvement of object detection by updating the recursive tracker in which all features are tracked jointly. The approach has been evaluated on four patient databases, providing an absolute mean displacement error less than 10 pixels ≈ 2mm). Its suitability for the TAVI procedure has been analyzed. PMID:24110703

  8. Stroke in Patients With Aortic Stenosis

    DEFF Research Database (Denmark)

    Greve, Anders Møller; Dalsgaard, Morten; Bang, Casper N;

    2014-01-01

    , and poststroke survival a secondary outcome. Cox models treating AVR as a time-varying covariate were adjusted for atrial fibrillation and congestive heart failure, hypertension, age≥75 years, diabetes mellitus, stroke/transient ischemic attack, vascular disease, age 65-74 years and female sex (CHA2DS2-VASc......BACKGROUND AND PURPOSE: There are limited data on risk stratification of stroke in aortic stenosis. This study examined predictors of stroke in aortic stenosis, the prognostic implications of stroke, and how aortic valve replacement (AVR) with or without concomitant coronary artery bypass grafting...... influenced the predicted outcomes. METHODS: Patients with mild-to-moderate aortic stenosis enrolled in the Simvastatin and Ezetimibe in Aortic Stenosis (SEAS) study. Diabetes mellitus, known atherosclerotic disease, and oral anticoagulation were exclusion criteria. Ischemic stroke was the primary end point...

  9. Transcatheter aortic valve replacement in elderly patients

    Institute of Scientific and Technical Information of China (English)

    Dimytri Siqueira; Alexandre Abizaid; Magaly Arrais J.; Eduardo Sousa

    2012-01-01

    Aortic stenosis is the most common native valve disease, affecting up to 5% of the elderly population. Surgical aortic valve replacement reduces symptoms and improves survival, and is the definitive therapy in patients with symptomatic severe aortic stenosis. However, despite the good results of classic surgery, risk is markedly increased in elderly patients with co-morbidities. Transcatheter aortic valve replacement (TAVR) allows implantation of a prosthetic heart valve within the diseased native aortic valve without the need for open heart surgery and cardiopulmonary bypass, offering a new therapeutic option to elderly patients considered at high surgical risk or with contraindications to surgery. To date, several multicenter registries and a randomized trial have confirmed the safety and efficacy of TAVR in those patients. In this chapter, we review the background and clinical applications of TAVR in elderly patients.

  10. MICrocephaly, disproportionate pontine and cerebellar hypoplasia syndrome: A clinico-radiologic phenotype linked to calcium/calmodulin-dependent serine protein kinase gene mutation

    Directory of Open Access Journals (Sweden)

    Rashid Saleem

    2013-01-01

    Full Text Available MICrocephaly, disproportionate pontine and cerebellar hypoplasia (MICPCH syndrome, a rare X-linked disorder, generally seen in girls, is characterized by neurodevelopmental delay, microcephaly, and disproportionate pontine and cerebellar hypoplasia. It is caused by inactivating calcium/calmodulin-dependent serine protein kinase (CASK gene mutations. We report a 2-year-old girl with severe neurodevelopmental delay, microcephaly, minimal pontine hypoplasia, cerebellar hypoplasia, and normal looking corpus callosum, with whom the conventional cytogenetic studies turned out to be normal, and an array-comparative genomic hybridization (a-CGH analysis showed CASK gene duplication at Xp11.4. Our case highlights the importance of using clinico-radiologic phenotype to guide genetic investigation and it also confirms the role of a-CGH analysis in establishing the genetic diagnosis of MICPCH syndrome, when conventional cytogenetic studies are inconclusive.

  11. Focal dermal hypoplasia: report of a case with myelomeningocele, Arnold-Chiari malformation and hydrocephalus with a review of neurologic manifestations of Goltz syndrome.

    Science.gov (United States)

    Peters, Tess; Perrier, Renee; Haber, Richard M

    2014-01-01

    Focal dermal hypoplasia (Goltz syndrome, Online Mendelian Inheritance in Man [OMIM] 305600) is a rare X-linked dominant congenital disorder involving defects of mesodermal- and ectodermal-derived structures. It is associated with mutations in the PORCN gene, a regulator of Wnt signaling proteins. The phenotype is highly variable, although all describe characteristic skin findings as a primary diagnostic feature. To date there are few case reports of focal dermal hypoplasia associated with central nervous system abnormalities. We report the second case of focal dermal hypoplasia associated with myelomenigocele, Arnold-Chiari malformation and hydrocephalus and the first in a male. Genetic testing identified a novel mosaic three base pair deletion within the PORCN gene (c.853_855delACG). This case highlights the importance of neurological evaluation in focal dermal hypoplasia and consideration of other syndromes more commonly associated with central nervous system abnormalities. In this report we summarize the literature on neurological manifestations in Goltz syndrome. PMID:24387693

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

    Institute of Scientific and Technical Information of China (English)

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

    2013-01-01

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

  13. Comparison of accuracy of aortic root annulus assessment with cardiac magnetic resonance versus echocardiography and multidetector computed tomography in patients referred for transcatheter aortic valve implantation.

    Science.gov (United States)

    Pontone, Gianluca; Andreini, Daniele; Bartorelli, Antonio L; Bertella, Erika; Mushtaq, Saima; Gripari, Paola; Loguercio, Monica; Cortinovis, Sarah; Baggiano, Andrea; Conte, Edoardo; Beltrama, Virginia; Annoni, Andrea; Formenti, Alberto; Tamborini, Gloria; Muratori, Manuela; Guaricci, Andrea; Alamanni, Francesco; Ballerini, Giovanni; Pepi, Mauro

    2013-12-01

    The evaluation of the aortic root in patients referred for transcatheter aortic valve implantation is crucial. The aim of the present study was to compare the accuracy of cardiac magnetic resonance (CMR) evaluation of the aortic annulus (AoA) with transthoracic and transesophageal echocardiography and multidetector computed tomography (MDCT) in patients referred for transcatheter aortic valve implantation. In 50 patients, maximum diameter, minimum diameter and AoA, length of the left coronary, right coronary, and noncoronary aortic leaflets, degree (grades 1 to 4) of aortic leaflet calcification, and distance between AoA and coronary artery ostia were assessed. AoA maximum diameter, minimum diameter, and area by CMR were 26.4 ± 2.8 mm, 20.6 ± 2.3 mm, 449.8 ± 86.2 mm(2), respectively. The length of left coronary, right coronary, and noncoronary leaflets by CMR were 13.9 ± 2.2, 13.3 ± 2.1, and 13.4 ± 1.8 mm, respectively, whereas the score of aortic leaflet calcifications was 2.9 ± 0.8. Finally, the distances between AoA and left main and right coronary artery ostia were 16.1 ± 2.8 and 16.1 ± 4.4 mm, respectively. Regarding AoA area, transthoracic and transesophageal echocardiography showed an underestimation (p <0.01), with a moderate agreement (r: 0.5 and 0.6, respectively, p <0.01) compared with CMR. No differences and excellent correlation were observed between CMR and MDCT for all parameters (r: 0.9, p <0.01), except for aortic leaflet calcifications that were underestimated by CMR. In conclusion, aortic root assessment with CMR including AoA size, aortic leaflet length, and coronary artery ostia height is accurate compared with MDCT. CMR may be a valid imaging alternative in patients unsuitable for MDCT. PMID:24045059

  14. Screening for Abdominal Aortic Aneurysm

    OpenAIRE

    Linné, Anneli

    2014-01-01

    Abdominal Aortic Aneurysm (AAA) is a common disease with a prevalence of 1.5-2.0% in 65-year old men in Sweden. The risk of having AAA is increased with smoking, high age, family history of AAA and cardiovascular disease. Women have a lower prevalence (0.5%) and develop AAA later in life. An AAA seldom gives any symptom prior to rupture. Untreated rupture is associated with 100% mortality, while surgically treated rupture is associated with 25-70% mortality. Prophylactic sur...

  15. Optic nerve head analysis of superior segmental optic hypoplasia using Heidelberg retina tomography

    Directory of Open Access Journals (Sweden)

    Atsushi Miki

    2010-10-01

    Full Text Available Atsushi Miki1,2, Motohiro Shirakashi1, Kiyoshi Yaoeda1, Atsushi Fukushima1, Mineo Takagi1, Haruki Abe11Division of Ophthalmology and Visual Sciences, Niigata University Graduate School of Medical and Dental Sciences, Niigata, 2Department of Ophthalmology, Kawasaki Medical School, Okayama, JapanPurpose: To evaluate the optic disc characteristics of eyes with superior segmental optic hypoplasia (SSOH using the Heidelberg retina tomograph (HRT.Patients and methods: Thirteen eyes of 13 Japanese patients with SSOH were studied with the HRT (software version: 3.0. The group included six males and seven females, with a mean age of 34.7 years. Six optic disc parameters in the six sectors derived from the patients with SSOH were compared with those of 13 eyes of 13 normal controls. In addition, the diagnostic classification performance of the Frederick S Mikelberg (FSM discriminant function, glaucoma probability score (GPS, and Moorfields regression analysis (MRA were assessed.Results: When compared with normal subjects, many of the optic disc parameters were significantly altered in SSOH in the superior sectors. The area under the curve (AUC for the receiver operating characteristic was 0.932 for the rim area, 0.926 for the cup-to-disc area ratio, and 0.882 for the cup shape measure. Among the HRT parameters, the largest AUC (0.988 was found for the cup shape measure in the nasal superior segment. The proportion classified as outside normal limits by the FSM discriminant function was 92.3% (12 eyes. For GPS, six eyes (46.2% were classified as outside normal limits. For MRA, when borderline cases were considered test-negative or test-positive, 10 eyes (76.9% or 11 eyes (84.6% were classified as outside normal limits, respectively. The AUCs were 0.976 for the FSM discriminant function, 0.914 for the MRA overall classification, and 0.710 for the GPS overall classification.Conclusions: In eyes with SSOH, there is a significant thinning of the rim

  16. Aortic valve and ascending aortic root modeling from 3D and 3D+t CT

    Science.gov (United States)

    Grbic, Saša; Ionasec, Razvan I.; Zäuner, Dominik; Zheng, Yefeng; Georgescu, Bogdan; Comaniciu, Dorin

    2010-02-01

    Aortic valve disorders are the most frequent form of valvular heart disorders (VHD) affecting nearly 3% of the global population. A large fraction among them are aortic root diseases, such as aortic root aneurysm, often requiring surgical procedures (valve-sparing) as a treatment. Visual non-invasive assessment techniques could assist during pre-selection of adequate patients, planning procedures and afterward evaluation of the same. However state of the art approaches try to model a rather short part of the aortic root, insufficient to assist the physician during intervention planning. In this paper we propose a novel approach for morphological and functional quantification of both the aortic valve and the ascending aortic root. A novel physiological shape model is introduced, consisting of the aortic valve root, leaflets and the ascending aortic root. The model parameters are hierarchically estimated using robust and fast learning-based methods. Experiments performed on 63 CT sequences (630 Volumes) and 20 single phase CT volumes demonstrated an accuracy of 1.45mm and an performance of 30 seconds (3D+t) for this approach. To the best of our knowledge this is the first time a complete model of the aortic valve (including leaflets) and the ascending aortic root, estimated from CT, has been proposed.

  17. Aortic root size and prevalence of aortic regurgitation in elite strength trained athletes.

    Science.gov (United States)

    Babaee Bigi, Mohammad Ali; Aslani, Amir

    2007-08-01

    Athletes involved in mainly static or isometric exercise (e.g., weight lifting, power lifting, and bodybuilding) develop pressure overloads due to the high systemic arterial pressure found in this type of exercise. It is hypothesized that chronically elevated aortic wall tension in strength-trained athletes is associated with aortic dilatation and regurgitation. The aim of this study was to evaluate aortic root size and the prevalence of aortic regurgitation in elite strength-trained athletes. The cohort included 100 male athletes (mean age 22.1 +/- 3.6 years; all were finalists or medalists in the country) and 128 healthy age- and height-matched subjects (the control group). Aortic root diameters at end-diastole were measured at 4 locations: (1) the aortic annulus, (2) the sinuses of Valsalva, (3) the sinotubular junction, and (4) the maximal diameter of the proximal ascending aorta. Aortic root diameters at all levels were significantly greater in the strength-trained athletes (p 18 and 36 and 54 months), progressive enlargement was found at all aortic diameters. In conclusion, aortic root diameters in all segments of the aortic root were significantly greater in elite strength-trained athletes compared with an age- and height-matched population.

  18. Idiopathic abdominal cocoon syndrome with unilateral abdominal cryptorchidism and greater omentum hypoplasia in a young case of small bowel obstruction

    Science.gov (United States)

    Fei, Xiang; Yang, Hai-Rui; Yu, Peng-Fei; Sheng, Hai-Bo; Gu, Guo-Li

    2016-01-01

    Abdominal cocoon syndrome (ACS) is a rare cause of intestinal obstruction due to total or partial encapsulation of the small intestine by a fibrocollagenous membrane. Idiopathic ACS with abdominal cryptorchidism and greater omentum hypoplasia is even rarer clinically. We successfully treated a 26-year-old male case of small bowel obstruction with acute peritonitis. He was finally diagnosed with idiopathic ACS with unilateral abdominal cryptorchidism and greater omentum hypoplasia during exploratory laparotomy. He then underwent enterolysis, cryptorchidectomy, and appendectomy. He recovered gradually from the operations and early postoperative inflammatory ileus. There has been no recurrence of intestinal obstruction since the operation, and he is still in follow-up. We analyzed his clinical data and retrospectively reviewed the literature, and our findings may be helpful for the clinical diagnosis and treatment on ACS. PMID:27239122

  19. Giant Thoracic Aneurysm Following Valve Replacement for Bicuspid Aortic Valve.

    Science.gov (United States)

    Tran, Cao; Ul Haq, Ehtesham; Nguyen, Ngoc; Omar, Bassam

    2015-01-01

    Bicuspid aortic valve is a common congenital anomaly associated with aortopathy, which can cause aortic root dilatation, necessitating regular screening if the aortic root is > 4.0 cm. Despite the low absolute incidence of aortic complications associated with bicuspid aortic valve in the general population, the consequences of such complications for an individual patient can be devastating. Herein we propose a balanced algorithm that incorporates recommendations from the three major guidelines for follow-up imaging of the aortic root and ascending thoracic aorta in patients with a bicuspid aortic valve, maintaining the current recommendations with regard to surgical thresholds. PMID:26827748

  20. Restoration of incisal half with edge-up technique using ceramic partial crown in turner′s hypoplasia: A case report

    OpenAIRE

    Shreya Hegde; Kundabala, M

    2014-01-01

    This case report describes a rare treatment modality for Turner's hypoplasia done with a very conservative approach for the esthetic and functional problem of the defect. Diagnosis was made as Turner's hypoplasia of upper two central incisors with proximal caries. Treatment planning was done after considering many factors such as conservation of tooth structure, esthetics, occlusion and economy. Tooth preparation was done to receive Edge-up, all ceramic partial crowns for both the upper centr...

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

    Science.gov (United States)

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

    1998-01-01

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

  2. A sneeze: an unusual trigger for aortic dissection

    OpenAIRE

    Upadhyaya, Subramanya G N; Large, A.

    2013-01-01

    We describe a rare case of type B thoracic aortic dissection resulting from a forceful sneeze in a 57-year-old man. The presenting clinical features were not typical and consisted of pleuritic chest pain and breathlessness following a forceful sneeze. There was no haemodynamic compromise. The diagnosis was made incidentally when he underwent a CT pulmonary angiography as part of pulmonary embolism work-up.

  3. Clinical distinction between nasal optic disc hypoplasia (NOH and glaucoma with NOH-like temporal visual field defects

    Directory of Open Access Journals (Sweden)

    Hiroshi Ohguro

    2010-06-01

    Full Text Available Hiroshi Ohguro, Ikuyo Ohguro, Midori Tsuruta, Maki Katai, Sachie TanakaDepartment of Ophthalmology, Sapporo Medical University School of Medicine, JapanPurpose: To report on the clinically important differences between nasal optic hypoplasia (NOH and glaucoma with NOH-like temporal visual field defect (VFD.Method: Five NOH (four bilateral and one unilateral patients, three unilateral NOH patients with glaucoma, and two glaucoma patients with NOH-like temporal VFD were clinically characterized. Superior segmental optic nerve hypoplasia was also associated with glaucoma in one eye of a bilateral NOH case and the NOH eye of a unilateral NOH patient. Ocular manifestations including refractive errors, size, and appearances of the optic discs, retinal nerve fiber thickness (NFLT ascertained by optical coherence tomography (OCT, and VFD were examined.Results: Ophthalmic examinations revealing NOH showed high myopia at more than -5.0D, a small disc with nasal double-ring appearance, significantly decreased NFLT by OCT, and retinal nerve fiber layer defect in the corresponding nasal sector. Stationary temporal VFD varied from a slight depression of the peripheral isopters to wide sector defects. In contrast, two glaucoma patients with NOH-like temporal VFD showed several different clinical features, including mild myopia less than -5D, a normal size with glaucomatous disc cupping; a slight decrease in nasal NFLT and progression of temporal and other glaucomatous VFD.Conclusion: Careful evaluation of optic disc appearance and measurement of NFLT using OCT may help to distinguish between NOH and glaucoma with NOH-like temporal VFD.Keywords: nasal optic disc hypoplasia, glaucoma, temporal visual field defects, optical coherence tomography, superior segmental optic nerve hypoplasia

  4. Anterior maxillary segmental distraction in the treatment of severe maxillary hypoplasia secondary to cleft lip and palate

    Science.gov (United States)

    Li, Hongliang; Dai, Jiewen; Si, Jiawen; Zhang, Jianfei; Wang, Minjiao; Shen, Steve Guofang; Yu, Hongbo

    2015-01-01

    Anterior maxillary segmental distraction (AMSD) is an effective surgical procedure in the treatment of maxillary hypoplasia secondary to cleft lip and palate. Its unique advantage of preserving velopharyngeal function makes this procedure widely applied. In this study, the application of AMSD was described and its long-term stability was explored. Eight patients with severe maxillary hypoplasia secondary to CLP were included in this study. They were treated with AMSD using rigid external distraction (RED) device. Cephalometric analysis was performed twice at three time points for evaluation: before surgery (T1), after distraction (T2), and 2 years after treatment (T3). One-way analysis of variance was used to assess the differences statistically. All the distractions completed smoothly, and maxilla was distracted efficiently. The value of SNA, NA-FH, Ptm-A, U1-PP, overjet and PP (ANS-PNS) increased significantly after the AMSD procedure (P 0.05). Changes of palatopharyngeal depth and soft palatal length were insignificant. AMSD with RED device provided an effective way to correct maxillary hypoplasia secondary to CLP, extended the palatal and arch length, avoided damage on velopharyngeal closure function and reduced the relapse rate. It is a promising and valuable technique in this potentially complicated procedure. PMID:26629107

  5. Early Developmental Perturbations in a Human Stem Cell Model of MODY5/HNF1B Pancreatic Hypoplasia

    Directory of Open Access Journals (Sweden)

    Adrian Kee Keong Teo

    2016-03-01

    Full Text Available Patients with an HNF1BS148L/+ mutation (MODY5 typically exhibit pancreatic hypoplasia. However, the molecular mechanisms are unknown due to inaccessibility of patient material and because mouse models do not fully recapitulate MODY5. Here, we differentiated MODY5 human-induced pluripotent stem cells (hiPSCs into pancreatic progenitors, and show that the HNF1BS148L/+ mutation causes a compensatory increase in several pancreatic transcription factors, and surprisingly, a decrease in PAX6 pancreatic gene expression. The lack of suppression of PDX1, PTF1A, GATA4, and GATA6 indicates that MODY5-mediated pancreatic hypoplasia is mechanistically independent. Overexpression studies demonstrate that a compensatory increase in PDX1 gene expression is due to mutant HNF1BS148L/+ but not wild-type HNF1B or HNF1A. Furthermore, HNF1B does not appear to directly regulate PAX6 gene expression necessary for glucose tolerance. Our results demonstrate compensatory mechanisms in the pancreatic transcription factor network due to mutant HNF1BS148L/+ protein. Thus, patients typically develop MODY5 but not neonatal diabetes despite exhibiting pancreatic hypoplasia.

  6. Detection of increased frequency of thyroid hypoplasia in subjects irradiated in utero as the results of Chernobyl catastrophe

    Energy Technology Data Exchange (ETDEWEB)

    Drozd, V.; Danilova, L.; Lushchyk, M.; Leonova, T.; Platonova, T. [International Fund Arnica, Minsk (Belarus); Grigorovich, A.; Sivuda, V. [Brest Regional Endocrinological Dispensary, Brest (Belarus); Branovan, I. [Chernobyl Project, New-York (United States); Biko, I.; Reiners, C. [Clinic and Policlinic of Nuclear Medicine, University of Wurzburg, Wursburg (Germany)

    2012-07-01

    For the 24 years passed after the Chernobyl catastrophe a significant experience in estimation of medical consequences of thyroid irradiation among Belarus patients had been accumulated. The aim of our screening of ultrasonic examination was the detection of the thyroid hypoplasia prevalence in the regions affected with radionuclide fallout. Since 2004 to 2007 thyroid ultrasound with volume estimation was performed in 3311 Belarus subjects, living on the areas of Brest region with the different contamination rate density. Examined subjects were divided in 3 groups: 1) irradiated at the age of 1 to 3 years old at the moment of Chernobyl catastrophe, 2) irradiated in utero, and 3) born after the catastrophe. It was revealed that thyroid hypoplasia was detected in 3% of group 1 (out of 1876 persons), in 5, 8% of group 2 (out of 503 persons, P<0.05) and in 1, 7% of the third group (out of 932 persons). The separation of the irradiated in utero subjects (group 2) to subgroups in dependence of the gestation period, showed the highest prevalence of thyroid hypoplasia among the irradiated in the first trimester of gestation: 7, 7% (P<0.05), in the second trimester: 5, 3%, in the third trimester: 4, 7%

  7. Simulation of bifurcated stent grafts to treat abdominal aortic aneurysms (AAA)

    Science.gov (United States)

    Egger, J.; Großkopf, S.; Freisleben, B.

    2007-03-01

    In this paper a method is introduced, to visualize bifurcated stent grafts in CT-Data. The aim is to improve therapy planning for minimal invasive treatment of abdominal aortic aneurysms (AAA). Due to precise measurement of the abdominal aortic aneurysm and exact simulation of the bifurcated stent graft, physicians are supported in choosing a suitable stent prior to an intervention. The presented method can be used to measure the dimensions of the abdominal aortic aneurysm as well as simulate a bifurcated stent graft. Both of these procedures are based on a preceding segmentation and skeletonization of the aortic, right and left iliac. Using these centerlines (aortic, right and left iliac) a bifurcated initial stent is constructed. Through the implementation of an ACM method the initial stent is fit iteratively to the vessel walls - due to the influence of external forces (distance- as well as balloonforce). Following the fitting process, the crucial values for choosing a bifurcated stent graft are measured, e.g. aortic diameter, right and left common iliac diameter, minimum diameter of distal neck. The selected stent is then simulated to the CT-Data - starting with the initial stent. It hereby becomes apparent if the dimensions of the bifurcated stent graft are exact, i.e. the fitting to the arteries was done properly and no ostium was covered.

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

    Directory of Open Access Journals (Sweden)

    Alexiou Christos

    2008-01-01

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

  9. iTRAQ proteomic analysis of extracellular matrix remodeling in aortic valve disease.

    Science.gov (United States)

    Martin-Rojas, Tatiana; Mourino-Alvarez, Laura; Alonso-Orgaz, Sergio; Rosello-Lleti, Esther; Calvo, Enrique; Lopez-Almodovar, Luis Fernando; Rivera, Miguel; Padial, Luis R; Lopez, Juan Antonio; de la Cuesta, Fernando; Barderas, Maria G

    2015-01-01

    Degenerative aortic stenosis (AS) is the most common worldwide cause of valve replacement. The aortic valve is a thin, complex, layered connective tissue with compartmentalized extracellular matrix (ECM) produced by specialized cell types, which directs blood flow in one direction through the heart. There is evidence suggesting remodeling of such ECM during aortic stenosis development. Thus, a better characterization of the role of ECM proteins in this disease would increase our understanding of the underlying molecular mechanisms. Aortic valve samples were collected from 18 patients which underwent aortic valve replacement (50% males, mean age of 74 years) and 18 normal control valves were obtained from necropsies (40% males, mean age of 69 years). The proteome of the samples was analyzed by 2D-LC MS/MS iTRAQ methodology. The results showed an altered expression of 13 ECM proteins of which 3 (biglycan, periostin, prolargin) were validated by Western blotting and/or SRM analyses. These findings are substantiated by our previous results demonstrating differential ECM protein expression. The present study has demonstrated a differential ECM protein pattern in individuals with AS, therefore supporting previous evidence of a dynamic ECM remodeling in human aortic valves during AS development. PMID:26620461

  10. The influence of the aortic valve angle on the hemodynamic features of the thoracic aorta

    Science.gov (United States)

    Ha, Hojin; Kim, Guk Bae; Kweon, Jihoon; Lee, Sang Joon; Kim, Young-Hak; Kim, Namkug; Yang, Dong Hyun

    2016-08-01

    Since the first observation of a helical flow pattern in aortic blood flow, the existence of helical blood flow has been found to be associated with various pathological conditions such as bicuspid aortic valve, aortic stenosis, and aortic dilatation. However, an understanding of the development of helical blood flow and its clinical implications are still lacking. In our present study, we hypothesized that the direction and angle of aortic inflow can influence helical flow patterns and related hemodynamic features in the thoracic aorta. Therefore, we investigated the hemodynamic features in the thoracic aorta and various aortic inflow angles using patient-specific vascular phantoms that were generated using a 3D printer and time-resolved, 3D, phase-contrast magnetic resonance imaging (PC-MRI). The results show that the rotational direction and strength of helical blood flow in the thoracic aorta largely vary according to the inflow direction of the aorta, and a higher helical velocity results in higher wall shear stress distributions. In addition, right-handed rotational flow conditions with higher rotational velocities imply a larger total kinetic energy than left-handed rotational flow conditions with lower rotational velocities.

  11. Case Report: En Bloc Resection of Pancoast Tumor with Adjuvant Aortic Endograft and Chemoradiation

    Science.gov (United States)

    Lu, Tony; Fischer, Uwe M.; Marco, Rex A.; Naoum, Joseph J.; Reardon, Michael J.; Lumsden, Alan B.; Blackmon, Shanda H.; Davies, Mark G.

    2015-01-01

    “Pancoast” tumors frequently require a multidisciplinary approach to therapy and are still associated with high morbidity and mortality. Due to their sensitive anatomic location, complex resections and chemoradiation regimens are typically required for treatment. Those with signs of aortic invasion pose an even greater challenge, given the added risks of cardiopulmonary bypass for aortic resection and interposition. Placement of an aortic endograft can facilitate resection if the tumor is in close proximity to or is invading the aorta. Prophylactic endografting to prevent radiation-associated aortic rupture has also been described. This case describes a 60-year-old female who presented with a stage IIIa left upper lobe undifferentiated non-small-cell carcinoma encasing the subclavian artery with thoracic aorta and bony invasion. Following carotid-subclavian bypass with Dacron, en bloc resection of the affected lung, ribs, and vertebral bodies was performed. The aorta was prophylactically reinforced with a Gore TAG thoracic endograft prior to adjuvant chemoradiation. The patient remains disease-free at more than 5 years follow-up after completing her treatment course. Endovascular stenting with subsequent chemoradiation may prove to be a viable alternative to palliation or open operative management and prevention of aortic injury during tumor resection and/or adjuvant therapy in select patients with aortic involvement. PMID:26306134

  12. The Placental Distal Villous Hypoplasia Pattern: Interobserver Agreement and Automated Fractal Dimension as an Objective Metric.

    Science.gov (United States)

    Mukherjee, Anika; Chan, Adrian D C; Keating, Sarah; Redline, Raymond W; Fritsch, Michael K; Machin, Geoffrey A; Cornejo-Palma, Daniel; de Nanassy, Joseph; El-Demellawy, Dina; von Dadelszen, Peter; Benton, Samantha J; Grynspan, David

    2016-01-01

    The distal villous hypoplasia (DVH) pattern is a placental correlate of fetal growth restriction. Because the pattern seems to involve less complexity than do appropriately developed placental villi, we postulated that it may be associated with lower fractal dimension-a mathematical measure of complexity. Our study objectives were to evaluate interobserver agreement related to the DVH pattern among expert pathologists and to determine whether pathologist classification of DVH correlates with fractal dimension. A study set of 30 images of placental parenchyma at ×4 magnification was created by a single pathologist from a digital slide archive. The images were graded for the DVH pattern according to pre-specified definitions and included 10 images graded as "no DVH" (grade  =  0), 10 with mild to moderate DVH (grade  =  1), and 10 with severe DVH (grade  =  2). The images were randomly sorted and shown to a panel of 4 international experts who similarly graded the images for DVH. Weighted kappas were calculated. For each image, fractal dimension was calculated by the Box Counting method. The correlation coefficient between (1) the averaged DVH scores obtained by the 5 pathologists and (2) fractal dimension was calculated. The mean weighted kappa score among the observers was 0.59 (range: 0.42-0.70). The correlation coefficient between fractal dimension and the averaged DVH score was -0.915 (P fractal dimension and represents an objective measure for DVH.

  13. Iron as the Key Modulator of Hepcidin Expression in Erythroid Antibody-Mediated Hypoplasia

    Directory of Open Access Journals (Sweden)

    J. C. Fernandes

    2014-01-01

    Full Text Available Erythroid hypoplasia (EH is a rare complication associated with recombinant human erythropoietin (rHuEPO therapies, due to development of anti-rHuEPO antibodies; however, the underlying mechanisms remain poorly clarified. Our aim was to manage a rat model of antibody-mediated EH induced by rHuEPO and study the impact on iron metabolism and erythropoiesis. Wistar rats treated during 9 weeks with a high rHuEPO dose (200 IU developed EH, as shown by anemia, reduced erythroblasts, reticulocytopenia, and plasmatic anti-rHuEPO antibodies. Serum iron was increased and associated with mRNA overexpression of hepatic hepcidin and other iron regulatory mediators and downregulation of matriptase-2; overexpression of divalent metal transporter 1 and ferroportin was observed in duodenum and liver. Decreased EPO expression was observed in kidney and liver, while EPO receptor was overexpressed in liver. Endogenous EPO levels were normal, suggesting that anti-rHuEPO antibodies blunted EPO function. Our results suggest that anti-rHuEPO antibodies inhibit erythropoiesis causing anemia. This leads to a serum iron increase, which seems to stimulate hepcidin expression despite no evidence of inflammation, thus suggesting iron as the key modulator of hepcidin synthesis. These findings might contribute to improving new therapeutic strategies against rHuEPO resistance and/or development of antibody-mediated EH in patients under rHuEPO therapy.

  14. Results of surgery for aortic regurgitation due to aortic valve prolapse.

    Directory of Open Access Journals (Sweden)

    Shigenobu,Masaharu

    1988-12-01

    Full Text Available The clinical, hemodynamic and pathological findings of 13 patients with aortic regurgitation due to aortic valve prolapse caused by advanced myxomatous degeneration were evaluated. Eleven patients showed a favorable outcome with no complications resulting from surgery. One patient died from aortic dissection, and another died suddenly from an unknown cause. Five patients had mitral valve prolapse as a complication. Ten patients (77% had a long-standing history of hypertension. Twelve patients (92% were male. None of the patients had the stigmata of Marfan's syndrome. All patients had marked myxomatous degeneration of the aortic valves without any inflammatory changes. Two patients showed microcalcification; 7 demonstrated moderate fibrosis. Five patients showed severe fragility of the cusps which appeared redundant, gelatinous and softened by degenerative changes. Myxomatous degeneration of the aortic valve is not rare, and, in fact, it may be one of the most common pathologic and clinical entities associated with pure aortic insufficiency.

  15. Transcatheter Versus Surgical Aortic Valve Replacement in Patients With Severe Aortic Valve Stenosis

    DEFF Research Database (Denmark)

    Thyregod, Hans Gustav; Steinbrüchel, Daniel Andreas; Ihlemann, Nikolaj;

    2015-01-01

    BACKGROUND: Transcatheter aortic valve replacement (TAVR) is an option in certain high-risk surgical patients with severe aortic valve stenosis. It is unknown whether TAVR can be safely introduced to lower-risk patients. OBJECTIVES: The NOTION (Nordic Aortic Valve Intervention Trial) randomized...... clinical trial compared TAVR with surgical aortic valve replacement (SAVR) in an all-comers patient cohort. METHODS: Patients ≥ 70 years old with severe aortic valve stenosis and no significant coronary artery disease were randomized 1:1 to TAVR using a self-expanding bioprosthesis versus SAVR. The primary...... conduction abnormalities requiring pacemaker implantation, larger improvement in effective orifice area, more total aortic valve regurgitation, and higher New York Heart Association functional class at 1 year. SAVR-treated patients had more major or life-threatening bleeding, cardiogenic shock, acute kidney...

  16. Comparative study of calcified changes in aortic valvular diseases.

    Science.gov (United States)

    Togashi, Mayuko; Tamura, Koichi; Masuda, Yukinari; Fukuda, Yuh

    2008-06-01

    Calcification of the aortic valve leads to stenosis or regurgitation or both. To clarify the mechanism of heart valve calcification, comparative studies using histological and ultrastructural examinations were performed of calcified aortic valves. These valves were obtained at valve replacement surgery from 11 patients with rheumatic aortic valvular disease (RAVD), 10 patients with degenerative aortic valve disease (DAVD), and 10 patients with congenitally bicuspid aortic valves (CBAV). For electron microscopic study, 5 cases were selected from each group. In RAVD, histological examination revealed calcification in a degenerated amorphous area at the center of fibrous thickened regions and in laminar fibrous thickened areas near the valve surface. In DAVD, calcification was observed mainly in the fibrosa near the valve ring. In CBAV, basic pathological changes were similar to those in DAVD; however, additional severe calcification of the raphe was observed, if the raphe was present. Ultrastructural examinations showed deposition of electron-dense materials in two patterns in all three groups; one pattern was observed in the interfibrillar spaces of collagen fibrils, and the other pattern was widespread macular deposition unrelated to the preexisting structure. In RAVD, microfibril-like fibrillar structures were found in the areas of deposition of electron-dense materials. These findings suggest that newly formed connective tissue degraded and became necrotic because of nutritional deprivation, especially in the thickened central area, causing calcium deposition. In DAVD and CBAV, numerous lipid vacuoles were found in the electron-dense deposition areas similar to lipid deposition in aortic atherosclerosis. Localized calcium deposition in the fibrosa suggests that the stress of valvular motion and pressure load induces sclerotic changes with the degeneration of collagen fibers, providing a core for calcification. In CBAV, the raphe was the main location of

  17. 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.

  18. Anatomics Knowledges of Aortic Root in the Transcatheter Aortic Valve Implantation.

    OpenAIRE

    Marcelo Cerezo; Omar Bertani; Gisela Panciroli; Sebastián Duhalde; Karina Ferreira; Luciano Honaine

    2010-01-01

    The Aortic Valve is a valvular system that bears different high pressure forces. It takes part of a complex structure called Aortic Root. Nowadays, anatomic knowledge has taken a preponderant role, due to the use of the Transcatether Aortic Valve Implant (TAVI). To describe the patient´s characteristics which exclude them of the TAVI for anatomic reasons in cadaveric dissections and transthoracic echocardiograms. A descriptive retrospective analysis of 67 individuals was performed and divided...

  19. Infective endocarditis complicated by aortic graft infection and osteomyelitis: case report and review of literature

    Directory of Open Access Journals (Sweden)

    Zouein E

    2012-07-01

    Full Text Available Elie Zouein,1 Robert Wetz,1 Neville Mobarakai,1 Samer Hassan,1 Iris Tong21Department of Medicine, Staten Island University Hospital, New York, NY USA; 2Department of Medicine, Warren Alpert Medical School of Brown University, Providence, RI, USAAbstract: Primary aortic graft infection early after aortic graft insertion is well described in the literature. Here, we present a unique case of late aortic graft infection 5 years after insertion secondary to mitral valve endocarditis, resulting from cellulitis in a patient with severe venous varicosities. A 63-year-old male presented for severe low back pain, constipation, and low-grade fever. An abdominal computed tomography scan with oral and intravenous contrast showed a normal spine and urinary tract. Blood and urine cultures, done at the same time, grew Staphylococcus aureus. A transesophageal echocardiogram confirmed the diagnosis of endocarditis. Subsequently, a gallium scan showed increased uptake in the vertebral bodies, aortic graft, left patella, and left ankle. After 3 months of antibiotic therapy, the patient's low back pain resolved with normalization of his laboratory values. He remained free of infection at a 2-year follow-up. We reviewed the literature concerning the atypical presentation of infective endocarditis, with a focus on distant metastases at initial presentation, such as osteomyelitis and aortic graft infection, as well as the different treatment modalities. This report describes successful medical treatment with intravenous followed by oral antibiotics for an infected endovascular graft without any surgical intervention.Keywords: endocarditis, osteomyelitis, aortic graft infection, septic emboli, endovascular abdominal aortic aneurysm repair (EVAR

  20. Branched and fenestrated options to treat aortic arch aneurysms.

    Science.gov (United States)

    Maurel, Blandine; Mastracci, Tara M; Spear, Rafaelle; Hertault, Adrien; Azzaoui, Richard; Sobocinski, Jonathan; Haulon, Stephan

    2016-10-01

    Conventional surgical repair of aortic arch aneurysms using cardiopulmonary bypass and hypothermic circulatory arrest remains the gold standard, however it is associated with a substantial mortality and morbidity rate, especially in the elderly. Hybrid techniques avoid aortic cross-clamping and circulatory arrest, but are of limited use and are only applicable to selected patients. The development of new devices to treat aortic arch aneurysms endovascularly has the potential to offer a treatment modality to patients unfit for an open repair. We present the challenges specific to endovascular arch repair based on our experience and the literature available from the first experience in 1999 to the third generation graft currently commonly used. Following an initial learning curve associated with the use of the third generation arch branch device, along with careful patient selection and operator experience, early results are promising. Technical success was achieved in all cases, there was no early mortality and strokes were noted in 11%. As with branched and fenestrated technology for thoracoabdominal aneurysm repair, the use of total endovascular repair for arch pathology will require an evolution in endovascular practice and device design. However, at present, the early use of the latest generation device offers a novel approach to patients who previously had no surgical options. PMID:27332680

  1. Aortic aneurysm and diverticulum of Kommerell: a dreadful concomitance

    Directory of Open Access Journals (Sweden)

    Fernando Peixoto Ferraz de Campos

    2012-12-01

    Full Text Available First described in 1936, the diverticulum of Kommerell (DOK is a dilatation of the proximal segment of an aberrant subclavian artery. Appearing more frequently in the left-sided aortic arch, the aberrant right subclavian artery passes behind the esophagus toward the right arm, causing symptoms in the minority of cases. Diagnosis is generally incidental with this pattern. When symptomatic, dysphagia, respiratory symptoms, hoarseness, chest pain, and upper limb ischemia are the most common complaints. Although debatable, the origin of DOK is accepted as being degenerative or congenital. The degenerative condition is normally associated with atherosclerosis and occurs more frequently after the age of 50 years with no gender predominance. Complications may be life threatening and are more commonly related to the diverticulum aneurysm or when associated with aortic diseases such as aneurysms or dissection. The authors present a case of a 67-year-old male with a history of acute chest pain, neurological disturbances, and hypertensive crisis. The diagnostic workup revealed an aortic arch aneurysm with intramural hematoma and a diverticulum aneurysm of Kommerell. Treatment was conservative at first. The patient presented a satisfactory outcome and was referred to an outpatient clinic for follow up and further therapeutic consolidation.

  2. Management of Traumatic Aortic and Splenic Rupture in a Patient With Ascending Aortic Aneurysm.

    Science.gov (United States)

    Topcu, Ahmet Can; Ciloglu, Ufuk; Bolukcu, Ahmet; Dagsali, Sabri

    2016-08-01

    Traumatic aortic rupture is rupture of all or part of the aortic wall, mostly resulting from blunt trauma to the chest. The most common site of rupture is the aortic isthmus. Traumatic rupture of the ascending aorta is rare. A 62-year-old man with a family history of ascending aortic aneurysm was referred to our hospital after a motor vehicle accident. He had symptoms of cardiogenic shock. A contrast-enhanced computed tomographic scan revealed rupture of the proximal ascending aorta and an ascending aortic aneurysm with a diameter of 55 mm at the level of the sinuses of Valsalva. Transthoracic echocardiography at the bedside revealed severe aortic valvular insufficiency. We performed a successful Bentall procedure. During postoperative recovery, the patient experienced a cerebrovascular accident. Transesophageal echocardiography did not reveal thrombosis of the mechanical prosthesis. The patient's symptoms resolved in time, and he was discharged from the hospital on postoperative day 47 without any sequelae. He has been symptom free during a 6-month follow-up period. We suggest that individuals who have experienced blunt trauma to the chest and have symptoms of traumatic aortic rupture and a known medical history of ascending aortic aneurysm should be evaluated for a rupture at the ascending aorta and the aortic isthmus. PMID:27449463

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

    DEFF Research Database (Denmark)

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

    2015-01-01

    endovascular aneurysm repair (TEVAR) has changed and extended management options in thoracic aorta disease, including in those patients deemed unfit or unsuitable for open surgery. Accordingly, transcatheter aortic valve replacement (TAVR) is increasingly used to treat patients with symptomatic severe aortic......An extensive thoracic aortic aneurysm (TAA) is a potentially life-threatening condition and remains a technical challenge to surgeons. Over the past decade, repair of aortic arch aneurysms has been accomplished using both hybrid (open and endovascular) and totally endovascular techniques. Thoracic...

  4. 局灶性真皮发育不全一例%A case of focal dermal hypoplasia

    Institute of Scientific and Technical Information of China (English)

    吴侃; 张韡; 黄莹雪; 胡彬; 孙建方

    2014-01-01

    患者男,17岁,出生时即出现多发条纹状红色斑片和丘疹,红色斑片菲薄易破,愈后遗留色素减退斑片.右手第1、2指指甲发育不全.随年龄增长逐渐出现泛发线状或漩涡状色素沉着条纹,部分黄红色丘疹增大形成斑块.于儿童期始逐渐出现多发的乳头瘤样皮损,以口周为著.皮肤科检查:全身泛发线状或漩涡状的色素沉着斑片,以躯干为著,其间散在色素减退斑片与黄红色斑块.口周、下颌及右腘窝多发乳头瘤样皮损.皮损组织病理检查:表皮角化不全,真皮厚度显著变薄,真皮乳头层血管增多,皮下脂肪层上移.诊断:局灶性真皮发育不全.%A 17-year-old boy presented with multiple striated erythema and papules at birth.The erythema was fragile,easily damaged,and often healed leaving hypopigmented patches.The right first and second finger nails were hypoplastic.Generalized linear or whorled hyperpigmented patches gradually developed,and some yellow-pink papules enlarged into plaques with age.Multiple papillomatous papules appeared during childhood,which were mainly distributed in the perioral region.Skin examination revealed widespread linear or whorled hyperpigmented patches intermingled with scattered hypopigmented patches and yellow-pink plaques,which were mainly located in the trunk.Multiple papillomatous papules were observed in the perioral region,mandibular region,and right popliteal fossa.Skin biopsy showed epidermal parakeratosis,diminished dermal thickness,increased blood vessel density in dermal papillae,upward migration of subcutaneous fat layer known as "fat herniation".A diagnosis of focal dermal hypoplasia was made.

  5. Type B Aortic Dissection: Management Updates

    Directory of Open Access Journals (Sweden)

    Naghmeh Moshtaghi

    2009-12-01

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

  6. Valve selection in aortic valve endocarditis

    Science.gov (United States)

    Zubrytska, Yana

    2016-01-01

    Aortic prosthetic valve endocarditis (PVE) is a potentially life-threatening disease. Mortality and incidence of infective endocarditis have been reduced in the past 30 years. Medical treatment of aortic PVE may be successful in patients who have a prompt response after antibiotic treatment and who do not have prosthetic dysfunction. In advanced stages, antibiotic therapy alone is insufficient to control the disease, and surgical intervention is necessary. Surgical treatment may be lifesaving, but it is still associated with considerable morbidity and mortality. The aim of surgery is to perform a radical excision of all infected and necrotic tissue, reconstruction of the left ventricle outflow tract, and replacement of the aortic valve. There is no unanimous consensus on which is the optimal prosthesis to implant in this context, and several surgical techniques have been suggested. We aim to analyze the efficacy of the surgical treatment and discuss the issue of valve selection in patients with aortic valve endocarditis.

  7. Dissecting aortic aneurysm in maintenance hemodialysis patients

    Directory of Open Access Journals (Sweden)

    Ounissi M

    2009-01-01

    Full Text Available The dissecting aortic aneurysm (DAA is a rare pathology that may result in fatal outcome. We report follow up of three cases of DAA patients undergoing maintenance hemo-dialysis who were managed conservatively.

  8. Circulating CD14+ monocytes in patients with aortic stenosis

    Institute of Scientific and Technical Information of China (English)

    Sara Shimoni; Valery Meledin; Iris Bar; Jacob Fabricant; Gera Gandelman; Jacob George

    2016-01-01

    BackgroundCalcific aortic stenosis (AS) is an active process sharing similarities with atherosclerosis and chronic inflammation. The pathophysiology of AS is notable for three cardinal components: inflammation, fibrosis and calcification. Monocytes play a role in each of these processes. The role of circulating monocytes in AS is not clear. The aim of the present study was to study an association between cir-culating apoptotic and non apoptotic CD14+ monocytes and AS features.MethodsWe assessed the number of CD14+ monocytes and apoptotic monocytes in 54 patients with significant AS (aortic valve area 0.74 ± 0.27 cm2) and compared them to 33 patients with similar risk factors and no valvular disease. The level of CD14+ monocytes and apoptotic monocytes was assessed by flow cytometry.ResultsThere was no difference in the risk factor profile and known coronary or peripheral vascular diseases between patients with AS and controls.Pa-tients with AS exhibited increased numbers of CD14+ monocytes as compared to controls (9.9% ± 4.9%vs. 7.7% ± 3.9%,P= 0.03). CD14+ monocyte number was related to age and the presence and severity of AS. In patients with AS, both CD14+ monocytes and apoptotic mono-cytes were inversely related to aortic valve area.ConclusionsPatients with significant AS have increased number of circulating CD14+ monocytes and there is an inverse correlation between monocyte count and aortic valve area. These findings may suggest that inflammation is operative not only in early valve injury phase, but also at later developed stages such as calcification when AS is severe.

  9. Acute Right Coronary Ostial Stenosis during Aortic Valve Replacement

    Directory of Open Access Journals (Sweden)

    Sarwar Umran

    2012-01-01

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

  10. Inside human aortic stenosis: a proteomic analysis of plasma.

    Science.gov (United States)

    Gil-Dones, Félix; Darde, Verónica M; Alonso-Orgaz, Sergio; Lopez-Almodovar, Luis F; Mourino-Alvarez, Laura; Padial, Luis R; Vivanco, Fernando; Barderas, Maria G

    2012-02-16

    Valvular aortic stenosis (AS) produces a slowly progressive obstruction in left ventricular outflow track. For this reason, aortic valve replacement is warranted when the valvular stenosis is hemodinamically significant, becoming the most common worldwide cause of aortic valve surgery. Recent epidemiologic studies have revealed an association between degenerative AS and cardiovascular risk factors for atherosclerosis, althought reducing the exposure to such factors and statin therapies both fail to delay or reverse the pathology. Hence, a deeper understanding of the pathophysiology of this disease is required to identify appropriate preventive measures. A proteomic analysis of plasma will permit to know and identify the changes in protein expression induced by AS in this tissue. Using two-dimensional difference gel electrophoresis (2D-DIGE) followed by mass spectrometry (MS), we compared the crude (not pre-fractioned) and pre-fractioned plasma from AS patients and control subjects. We sought to identify plasma proteins whose expression is modified in AS. In addition we investigated if crude plasma presented some alterations in the more abundant proteins since to date, has never been studied before. We also further investigated the link between this disease and atherosclerosis with a view to identifying new potential markers and therapeutic targets. PMID:22178735

  11. Recently patented transcatheter aortic valves in clinical trials.

    Science.gov (United States)

    Neragi-Miandoab, Siyamek; Skripochnik, Edvard; Salemi, Arash; Girardi, Leonard

    2013-12-01

    The most widely used heart valve worldwide is the Edwards Sapien, which currently has 60% of the worldwide transcatheter aortic valve implantation (TAVI) market. The CoreValve is next in line in popularity, encompassing 35% of the worldwide TAVI market. Although these two valves dominate the TAVI market, a number of newer transcatheter valves have been introduced and others are in early clinical evaluation. The new valves are designed to reduce catheter delivery diameter, improve ease of positioning and sealing, and facilitate repositioning or removal. The most recent transcatheter valves for transapical use include Acurate TA (Symetis), Engager (Medtronic), and JenaValve the Portico (St Jude), Sadra Lotus Medical (Boston Scientific), and the Direct Flow Medical. These new inventions may introduce more effective treatment options for high-risk patients with severe aortic stenosis. Improvements in transcatheter valves and the developing variability among them may allow for more tailored approaches with respect to patient's anatomy, while giving operators the opportunity to choose devices they feel more comfortable with. Moreover, introducing new devices to the market will create a competitive environment among producers that will reduce high prices and expand availability. The present review article includes a discussion of recent patents related to Transcatheter Aortic Valves. PMID:24279506

  12. The role of gadolinium-enhanced MR imaging in the preoperative evaluation of inflammatory abdominal aortic aneurysm

    Energy Technology Data Exchange (ETDEWEB)

    Anbarasu, A.; McWilliams, R.G. [Department of Radiology, Royal Liverpool University Hospital, Prescot Street, Liverpool, L7 8XP (United Kingdom); Harris, P.L. [Department of Vascular Surgery, Royal Liverpool University Hospital, Prescot Street, Liverpool, L7 8XP (United Kingdom)

    2002-07-01

    Inflammatory abdominal aortic aneurysm is an uncommon variant of abdominal aortic aneurysms. Thorough preoperative imaging of the extent of the aneurysm and inflammation and the associated complications are crucial in the management of this condition. We report a case of inflammatory abdominal aortic aneurysm where, after the initial contrast-enhanced CT, gadolinium-enhanced MR imaging was used to define the true extent of the inflammation and differentiate inflammation from mural thrombus at the iliac extension of the aneurysm. The imaging appearances are presented and the impact of MR imaging on further surgical management options including endovascular repair are discussed. (orig.)

  13. Role of F-18 FDG PET/CT in the management of infected abdominal aortic aneurysm due to salmonella

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Seung Jin; Lee, Jin Soo; Cheong, Moon Hyun; Byun, Sung Su; Hyun, In Young [Inha University College of Medicine, Incheon (Korea, Republic of)

    2007-12-15

    We present a case of infected abdominal aortic aneurysm due to salmonella enteritidis. F-18 FDG PET/CT was performed to diagnosis and during follow-up after antibiotic treatment. Computed tomography (CT) is considered to be the best diagnostic imaging modality in infected aortic lesions. In this case, a combination of CT and FDG PET/CT provided accurate information for the diagnosis of infected abdominal aortic aneurysm. Moreover, FDG PET/CT made an important contribution of monitoring disease activity during antibiotic treatment.

  14. 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.

  15. Distortion of the CoreValve during transcatheter aortic valve-in-valve implantation due to valve dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Souteyrand, Geraud, E-mail: gsouteyrand@chu-clermontferrand.fr [Department of Cardiology, Gabriel Montpied Hospital, CHU Clermont-Ferrand, Clermont-Ferrand (France); ERIM-EA3295, University of Auvergne, Clermont-Ferrand (France); Wilczek, Krzysztof [Department of Cardiology, Medical University of Silesia, Silesian Centre for Herat Diseases, Zabrze (Poland); Innorta, Andrea; Camilleri, Lionel [Department of Cardiology, Gabriel Montpied Hospital, CHU Clermont-Ferrand, Clermont-Ferrand (France); ERIM-EA3295, University of Auvergne, Clermont-Ferrand (France); Chodor, Piotr [Department of Cardiology, Medical University of Silesia, Silesian Centre for Herat Diseases, Zabrze (Poland); Lusson, Jean-René; Motreff, Pascal [Department of Cardiology, Gabriel Montpied Hospital, CHU Clermont-Ferrand, Clermont-Ferrand (France); ERIM-EA3295, University of Auvergne, Clermont-Ferrand (France); Laborde, Jean-Claude [St. George' s Hospital, London (United Kingdom); Chabrot, Pascal; Durel, Nicolas [Department of Cardiology, Gabriel Montpied Hospital, CHU Clermont-Ferrand, Clermont-Ferrand (France); ERIM-EA3295, University of Auvergne, Clermont-Ferrand (France)

    2013-09-15

    Nowadays transcatheter aortic valve implantation (TAVI) is an accepted alternative to surgical aortic valve replacement for high-risk patients (pts). Successful TAVI procedures for failed aortic surgical bioprosthesis (TAV-in-SAV) have already been reported. In the presented two cases of TAV-in-SAV implantation a strut distortion of the stent was revealed on angiographic imaging and confirmed on control CT scan. In both procedures, a dislocation of the medtronic core valve (MCV) prosthesis during implantation led to valve retrieval, with a necessity of reloading it in the 18F introducer before subsequent implantation of the same valve in correct position.

  16. Anesthetic management of transcatheter aortic valve implantation

    OpenAIRE

    Annalisa Franco; Chiara Gerli; Laura Ruggeri; Fabrizio Monaco

    2012-01-01

    Transcatheter aortic valve implantation (TAVI) is an emergent technique for high-risk patients with aortic stenosis. TAVI poses significant challenges about its management because of the procedure itself and the population who undergo the implantation. Two devices are currently available and marketed in Europe and several other technologies are being developed. The retrograde transfemoral approach is the most popular procedure; nevertheless, it may not be feasible in patients with significant ...

  17. Navigation Technology in Endovascular Aortic Repair

    OpenAIRE

    Manstad-Hulaas, Frode

    2013-01-01

    A number of diseases can affect the aorta, and endovascular (minimally invasive) techniques can be used to treat many of these conditions. During endovascular aortic repair, different instruments, such as catheters (plastic tubes), metal wires and balloons are visualized by X-rays. Intermittent aortic injections of contrast medium improve the depiction of the aorta; however, contrast medium may damage kidney function in some patients, radiation can be harmful and X-ray images are 2- dimension...

  18. Surveillance intervals for small abdominal aortic aneurysms

    DEFF Research Database (Denmark)

    Bown, Matthew J; Sweeting, Michael J; Brown, Louise C;

    2013-01-01

    Small abdominal aortic aneurysms (AAAs [3.0 cm-5.4 cm in diameter]) are monitored by ultrasound surveillance. The intervals between surveillance scans should be chosen to detect an expanding aneurysm prior to rupture.......Small abdominal aortic aneurysms (AAAs [3.0 cm-5.4 cm in diameter]) are monitored by ultrasound surveillance. The intervals between surveillance scans should be chosen to detect an expanding aneurysm prior to rupture....

  19. Aortic dissection. Basic aspects and endovascular management

    International Nuclear Information System (INIS)

    Treatment of thoracic aortic pathology is complicated by the morbidity associated to the surgical procedure and to the frailty of an elderly and difficult population. Surgical operation in this kind of population frequently bears a significant incidence of death and long-term disability. In an effort to reduce the incidence of negative outcomes, minimally invasive techniques in the form of endovascular stenting have been introduced during the past decade. The technology, originally described by Parodi, and initially designed for its use in abdominal aortic aneurysms, has been adapted for the treatment of thoracic aortic aneurysms. Furthermore, an improved understanding of the pathophysiology and the natural history of thoracic aortic disease as well as the analysis of the outcomes have facilitated our treatment decisions in terms of the timing for an appropriate intervention. Treatment of thoracic aortic dissection using endovascular Stent is one of the more recent advances in this condition and is receiving increasing attention, as it is a less invasive alternative to an open surgical repair. Although this technology is still innovative, significant improvements have been made lately in the design and deployment of the endovascular Stent-grafts. These prostheses have been increasingly used to treat aneurysms, dissections and traumatic ruptures, as well as giant penetrating ulcers and intramural hematomas of the descending thoracic aorta with good early and mid-term outcomes. The rareness, complexity and severity of the pathology and the theoretically high risk of complications should render the surgeon extremely cautious especially with young patients. Conceptually, the endo luminal treatment in the acute phase seems to be the solution and will probably become a preferred therapy while technical refinement is under way. Worldwide experience is growing and with this a better understanding of the indications and limitations of this innovative therapy will be

  20. Effect of age on aortic atherosclerosis

    Institute of Scientific and Technical Information of China (English)

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

    2013-01-01

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

  1. Data on the circulating levels of endothelial microparticles are elevated in patients with bicuspid aortic valve and are related to aortic dilation

    Directory of Open Access Journals (Sweden)

    Josep M. Alegret

    2016-09-01

    Full Text Available The data included here support the research article “Circulating endothelial microparticles are elevated in bicuspid aortic valve (BAV disease and related to aortic dilation” (Alegret et al., 2016 [1] where circulating levels of platelet endothelial cell adhesion molecule (PECAM+ endothelial microparticles (EMPs were identified as a biological variable related to aortic dilation in patients with BAV disease. The data presented in this article are composed by four tables and one figure containing the clinical and echocardiographic characteristics of the patients (Alegret et al., 2016 [1] included in this study, and summarize the results of multivariate linear analyses. Furthermore, is also included a figure showing a representative flow cytometry dot plots and histograms used in PECAM+ EMPs quantification is also included.

  2. Cortical hypoplasia and ventriculomegaly of p73-deficient mice: Developmental and adult analysis.

    Science.gov (United States)

    Medina-Bolívar, Carolina; González-Arnay, Emilio; Talos, Flaminia; González-Gómez, Miriam; Moll, Ute M; Meyer, Gundela

    2014-08-01

    Trp73, a member of the p53 gene family, plays a crucial role in neural development. We describe two main phenotypic variants of p73 deficiency in the brain, a severe one characterized by massive apoptosis in the cortex leading to early postnatal death and a milder, non-/low-apoptosis one in which 50% of pups may reach adulthood using an intensive-care breeding protocol. Both variants display the core triad of p73 deficiency: cortical hypoplasia, hippocampal malformations, and ventriculomegaly. We studied the development of the neocortex in p73 KO mice from early embryonic life into advanced age (25 months). Already at E14.5, the incipient cortical plate of the p73 KO brains showed a reduced width. Examination of adult neocortex revealed a generalized, nonprogressive reduction by 10-20%. Area-specific architectonic landmarks and lamination were preserved in all cortical areas. The surviving adult animals had moderate ventricular distension, whereas pups of the early lethal phenotypic variant showed severe ventriculomegaly. Ependymal cells of wild-type ventricles strongly express p73 and are particularly vulnerable to p73 deficiency. Ependymal denudation by apoptosis and reduction of ependymal cilia were already evident in young mice, with complete absence of cilia in older animals. Loss of p73 function in the ependyma may thus be one determining factor for chronic hydrocephalus, which leads to atrophy of subcortical structures (striatum, septum, amygdala). p73 Is thus involved in a variety of CNS activities ranging from embryonic regulation of brain size to the control of cerebrospinal fluid homeostasis in the adult brain via maintenance of the ependyma.

  3. Chronic Stanford type A aortic dissection manifesting as systemic inflammatory disorder

    Directory of Open Access Journals (Sweden)

    Yana Kogan

    2016-05-01

    Full Text Available Typical presentation of type A aortic dissection usually encompasses severe acute chest pain, frequently radiating to the upper back, which is seen in more than 80% of the patients, while isolated back or abdominal pain have been repeatedly reported as the first manifestation of the disease as well. Occasionally, dyspnea due to acute aortic regurgitation, syncope, or stroke, secondary to obstruction of major cerebral vessels, have also been described at presentation of type A aortic dissection. Presentation of aortic dissection as a prolonged systemic illness with a number of nonspecific clinical and laboratory findings, such as low-grade fever, fatigue, malaise, weight loss, anemia, elevated acute phase response laboratory parameters, and absence of any of typical clinical features of the dissection syndrome has been only rarely reported. We describe a patient with type A chronic aortic dissection, manifesting as a systemic inflammatory disorder in the absence of acute chest syndrome. The diagnosis was made accidentally by computed tomography, ordered in the course of the regular work up. The patient underwent emergent surgery with resection and grafting of the dissected aorta. Pathological investigation demonstrated intense acute inflammation with neutrophilic infiltration in the vicinity of the intramural hemorrhage and necrosis, as well as granulation tissue with new vessels formation and collagen deposition in the outer media. The possible pathogenic mechanisms of the phenomenon are discussed. [Int J Res Med Sci 2016; 4(5.000: 1768-1771

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

    Science.gov (United States)

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

    2013-01-01

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

  5. Turner's syndrome associated with bicuspid aortic stenosis and dissecting aortic aneurysm

    OpenAIRE

    Slater, D N; Grundman, M. J.; Mitchell, L

    1982-01-01

    A case of Turner's syndrome is described associated with bicuspid aortic stenosis and fatal rupture of a thoracic dissecting aortic aneurysm. Histology of the aneurysm showed severe cystic medial necrosis. This association has not been previously described in the absence of coarctation.

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

    Institute of Scientific and Technical Information of China (English)

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

    2009-01-01

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

  7. ALTERNATIVE METHOD OF SURGICAL CORRECTION OF DISSECTING AORTIC ANEURYSMS WITH AORTIC INSUFFIECIENCY

    Directory of Open Access Journals (Sweden)

    M. L. Semenovsky

    2009-01-01

    Full Text Available Patients with dissecting aortic aneurysm and associated acute aortic insufficiency form a group of seriously ill patients with significant cardiac failure, generally involving other organs and systems. It justifies an attempt to reduce a surgical risk, by using more sparing procedures, including supracoronary replacement of the ascending aorta with its root reconstruction. The latter has been performed in 27 patients (mean 54,5 ± 2,1 years with dissecting aortic aneurysm and aortic valvular insufficiency in 1996 to 2009. The major etiological factor was atherosclerosis (88%/ Seventeen (63%, 6 (22,2% and 4 (16% had types I, IIA and II dissection, respectively. Overall hospital mortality was 11%. In late period, progressive aneurysm dissection needed reinterventions in 2 cases. The competence of the reconstructed aortic valve was satisfactory both just after surgery and throughout the follow-up. Indications for this option of chronic correction, surgical techniques, and immediate and long-term results are outlined. 

  8. Abdominal Aortic Diameter Is Increased in Males with a Family History of Abdominal Aortic Aneurysms

    DEFF Research Database (Denmark)

    Mejnert Jørgensen, Trine; Houlind, K; Green, A;

    2014-01-01

    OBJECTIVE: To investigate, at a population level, whether a family history of abdominal aortic aneurysm (AAA) is independently related to increased aortic diameter and prevalence of AAA in men, and to elucidate whether the mean aortic diameter and the prevalence of AAA are different between...... participants with male and female relatives with AAA. DESIGN: Observational population-based cross-sectional study. MATERIALS: 18,614 male participants screened for AAA in the VIVA-trial 2008-2011 with information on both family history of AAA and maximal aortic diameter. METHODS: Standardized ultrasound scan...... measurement of maximum antero-posterior aortic diameter. Family history obtained by questionnaire. Multivariate regression analysis was used to test for confounders: age, sex, smoking, comorbidity and medication. RESULTS: From the screened cohort, 569 participants had at least one first degree relative...

  9. Endovascular repair of ruptured abdominal aortic aneurysm

    Directory of Open Access Journals (Sweden)

    Šarac Momir

    2014-01-01

    Full Text Available Introduction. Rupture of an abdominal aortic aneurysm (AAA is a potentially lethal state. Only half of patients with ruptured AAA reach the hospital alive. The alternative for open reconstruction of this condition is endovascular repair (EVAR. We presented a successful endovascular reapir of ruptured AAA in a patient with a number of comorbidities. Case report. A 60-year-old man was admitted to our institution due to diffuse abdominal pain with flatulence and belching. Initial abdominal ultrasonography showed an AAA that was confirmed on multislice computed tomography scan angiography which revealed a large retroperitoneal haematoma. Because of patient’s comorbidites (previous surgery of laryngeal carcinoma and one-third laryngeal stenosis, arterial hypertension and cardiomyopathy with left ventricle ejection fraction of 30%, stenosis of the right internal carotid artery of 80% it was decided that endovascular repair of ruptured AAA in local anaesthesia and analgosedation would be treatment of choice. Endovascular grafting was achieved with aorto-bi-iliac bifurcated excluder endoprosthesis with complete exclusion of the aneurysmal sac, without further enlargment of haemathoma and no contrast leakage. The postoperative course of the patient was eventless, without complications. On recall examination 3 months after, the state of the patient was well. Conclusion. The alternative for open reconstruction of ruptured AAA in haemodynamically stable patients with suitable anatomy and comorbidities could be emergency EVAR in local anesthesia. This technique could provide greater chances for survival with lower intraoperative and postoperative morbidity and mortality, as shown in the presented patient.

  10. Measurement and determinants of infrarenal aortic thrombus volume

    Energy Technology Data Exchange (ETDEWEB)

    Golledge, Jonathan; Wolanski, Philippe [James Cook University, The Vascular Biology Unit, Townsville, Queensland (Australia); Townsville Hospital, Townsville, Queensland (Australia); Parr, Adam [James Cook University, The Vascular Biology Unit, Townsville, Queensland (Australia); Buttner, Petra [James Cook University, School of Public Health and Tropical Medicine, Townsville, Queensland (Australia)

    2008-09-15

    Intra-luminal thrombus has been suggested to play a role in the progression of abdominal aortic aneurysm (AAA). The aims of this study were twofold. Firstly, to assess the reproducibility of a computer tomography (CT)-based technique for measurement of aortic thrombus volume. Secondly, to examine the determinants of infrarenal aortic thrombus volume in a cohort of patients with aortic dilatation. A consecutive series of 75 patients assessed by CT angiography with maximum aortic diameter {>=}25 mm were recruited. Intra-luminal thrombus volume was measured by a semi-automated workstation protocol based on a previously defined technique to quantitate aortic calcification. Intra- and inter-observer reproducibility were assessed using correlation coefficients, coefficient of variation and Bland-Altman plots. Infrarenal aortic thrombus volume percentage was related to clinical, anatomical and blood characteristics of the patients using univariate and multivariate tests. Infrarenal aortic thrombus volume was related to the severity of aortic dilatation assessed by total aortic volume (r=0.87, P<0.0001) or maximum aortic diameter (r=0.74, P< 0.0001). We therefore examined the clinical determinates of aortic thrombus expressed as a percentage of total aortic volume. Aortic thrombus percentage was negatively correlated with serum high density lipoprotein (HDL, r=-0.31). By ordinal multiple logistic regression analysis serum HDL below median ({<=}1.2 mM) was associated with aortic thrombus percentage in the upper quartile adjusting for other risk factors (odds ratio 5.3, 95% CI 1.1-25.0). Infrarenal aortic thrombus volume can be measured reproducibly on CT. Serum HDL, which can be therapeutically raised, may play a role in discouraging aortic thrombus accumulation with implications in terms of delaying progression of AAA. (orig.)

  11. Aortic Dissection in a Healthy Male Athlete: A Unique Case with Comprehensive Literature Review

    Science.gov (United States)

    Singh, Balraj; Treece, Jennifer M.; Bhatheja, Samit; Lavine, Steven J.

    2016-01-01

    A young otherwise healthy 27-year-old male who has been using anabolic steroids for a long time developed Type I aortic dissection associated with heavy weightlifting. The patient did not have a recent history of trauma to the chest, no history of hypertension, and no illicit drug use. He presented with severe chest pain radiating to back and syncopal event with exertion. Initial vitals were significant for blood pressure of 80/50 mmHg, pulse of 80 beats per minute, respirations of 24 per minute, and oxygen saturation of 92% on room air. Physical exam was significant for elevated jugular venous pressure, muffled heart sounds, and cold extremities with diminished pulses in upper and absent pulses in lower extremities. Bedside echocardiogram showed aortic root dilatation and cardiac tamponade. STAT computed tomography (CT) scan of chest revealed dissection of ascending aorta. Cardiothoracic surgery was consulted and patient underwent successful repair of ascending aorta. Hemodynamic stress of weightlifting can predispose to aortic dissection. Aortic dissection is a rare but often catastrophic condition if not diagnosed and managed acutely. Although rare, aortic dissection needs to be in the differential when a young weightlifter presents with chest pain as a delay in diagnosis may be fatal. PMID:27738530

  12. Balloon aortic valvuloplasty as a bridge-to-decision in high risk patients with aortic stenosis: a new paradigm for the heart team decision making

    Science.gov (United States)

    Saia, Francesco; Moretti, Carolina; Dall'Ara, Gianni; Ciuca, Cristina; Taglieri, Nevio; Berardini, Alessandra; Gallo, Pamela; Cannizzo, Marina; Chiarabelli, Matteo; Ramponi, Niccolò; Taffani, Linda; Bacchi-Reggiani, Maria Letizia; Marrozzini, Cinzia; Rapezzi, Claudio; Marzocchi, Antonio

    2016-01-01

    Background Whilst the majority of the patients with severe aortic stenosis can be directly addressed to surgical aortic valve replacement (AVR) or transcatheter aortic valve implantation (TAVI), in some instances additional information may be needed to complete the diagnostic workout. We evaluated the role of balloon aortic valvuloplasty (BAV) as a bridge-to-decision (BTD) in selected high-risk patients. Methods Between 2007 and 2012, the heart team in our Institution required BTD BAV in 202 patients. Very low left ventricular ejection fraction, mitral regurgitation grade ≥ 3, frailty, hemodynamic instability, serious comorbidity, or a combination of these factors were the main drivers for this strategy. We evaluated how BAV influenced the final treatment strategy in the whole patient group and in each specific subgroup. Results Mean logistic European System for Cardiac Operative Risk Evaluation (EuroSCORE) was 23.5% ± 15.3%, age 81 ± 7 years. In-hospital mortality was 4.5%, cerebrovascular accident 1% and overall vascular complications 4% (0.5% major; 3.5% minor). Of the 193 patients with BTD BAV who survived and received a second heart team evaluation, 72.6% were finally deemed eligible for definitive treatment (25.4% for AVR; 47.2% for TAVI): 96.7% of patients with left ventricular ejection fraction recovery; 70.5% of patients with mitral regurgitation reduction; 75.7% of patients who underwent BAV in clinical hemodynamic instability; 69.2% of frail patients and 68% of patients who presented serious comorbidities. Conclusions Balloon aortic valvuloplasty can be considered as bridge-to-decision in high-risk patients with severe aortic stenosis who cannot be immediate candidates for definitive transcatheter or surgical treatment. PMID:27582761

  13. Moebius syndrome with macular hyperpigmentation, skeletal anomalies, and hypoplasia of pectoralis major muscle in an Egyptian child

    Directory of Open Access Journals (Sweden)

    Rabah M. Shawky

    2015-07-01

    Full Text Available We report a 4 month old female infant, 3rd in order of birth of the first cousin consanguineous parents. The patient has congenital right facial nerve palsy, with asymmetry of facial expression during crying and difficulty in swallowing. Associated anomalies include abnormal facial features, bilateral finger anomalies, bilateral talipes equinovarus, kyphoscoliosis, hypotonia, high frequency hearing loss. Bilateral macular hyperpigmentation was detected in our patient on fundus examination which was not reported previously in Moebius syndrome cases. In addition there is hypoplasia of the right pectoralis major muscle.

  14. Vasopressor mechanisms in acute aortic coarctation hypertension

    Directory of Open Access Journals (Sweden)

    Salgado H.C.

    1997-01-01

    Full Text Available Angiotensin II (ANG II and vasopressin (AVP act together with the mechanical effect of aortic constriction in the onset of acute aortic coarctation hypertension. Blockade of ANG II and AVP V1 receptors demonstrated that ANG II acts on the prompt (5 min rise in pressure whereas AVP is responsible for the maintenance (30-45 min of the arterial pressure elevation during aortic coarctation. Hormone assays carried out on blood collected from conscious rats submitted to aortic constriction supported a role for ANG II in the early stage and a combined role for both ANG II and AVP in the maintenance of proximal hypertension. As expected, a role for catecholamines was ruled out in this model of hypertension, presumably due to the inhibitory effect of the sinoaortic baroreceptors. The lack of afferent feedback from the kidneys for AVP release from the central nervous system in rats with previous renal denervation allowed ANG II to play the major role in the onset of the hypertensive response. Median eminence-lesioned rats exhibited a prompt increase in proximal pressure followed by a progressive decline to lower hypertensive levels, revealing a significant role for the integrity of the neuroaxis in the maintenance of the aortic coarctation hypertension through the release of AVP. In conclusion, the important issue raised by this model of hypertension is the likelihood of a link between some vascular territory - probably renal - below the coarctation triggering the release of AVP, with this vasoconstrictor hormone participating with Ang II and the mechanical effect of aortic constriction in the acute aortic coarctation hypertension

  15. Atherosclerotic Aortic Plaques Detected by Transesophageal Echocardiography

    Institute of Scientific and Technical Information of China (English)

    赵云; 朱文玲; 倪超; 郭丽琳; 曾勇; 方理刚

    2002-01-01

    Objective To evaluate the predictive value of atherosclerotic aortic plaques in coronary artery disease (CAD) Methods In 50patients with suspected coronary artery disease, transesophageal echocardiography was performed to examine their thoracic aortas 2 weeks before or after coronary angiography. In the cases of coronary angiography studied, stenosis of the coronary artery ≥ 50 % was considered to be due to coronary artery disease,whereas the thickness of the intima ≥ 1.3 mm was taken to be the criteria for the presence of an atherosclerotic aortic plaque on the transesophageal echocardiographic test. Results Among the 50 patients, 37 cases were diagnosed as CAD and 13 cases were considered to be normal. The plaques of the thoracic aorta were observed in 34cases in the CAD group and 3 cases in the normal group. The sensitivity and specificity of aortic plaques for CAD were 91.9 % and 76.9%, respectively. The positive and negative predictive values of the aortic plaques for CAD were 91.9% and 76.9%, respectively. The accuracy was 88.0%. 80 percent of the patients with single- yes sel disease had thoracic aortic plaques, 92 percent of the patients with two-vessel disease and 100 percent of the patients with three-vessel disease had thoracic aortic plaques. There was a significant difference in the thickness of aortic intimas between the normal group and the CAD group. Conclusions Detectingatherosclerotic plaques in the thoracic aorta with transesophageal echocardiography may be of great value in predicting the presence and extent of coronary artery disease.

  16. A Rare Cause of Foot Pain in the Emergency Room: an Aortic Dissection Case

    Directory of Open Access Journals (Sweden)

    Korkut S et al.

    2010-09-01

    Full Text Available Aortic dissection is a fatal condition that can originate from any point of the aorta between the root of the aorta and iliac bifurcation so it necessitates early diagnosis and treatment. The most common sign is pain and it may appear in the chest, neck, back or interscapular area. Additionally, it may present with anxiety, syncope or high blood pressure. In this article, we aimed to present a 46 year old female patient admitted to our emergency department due to paresthesia and pain in the right foot and took the diagnosis of DeBakey Type 1 aortic dissection.

  17. [Infrarenal aortic pseudo-aneurism due to Coxiella burnetii].

    Science.gov (United States)

    Paumier, A; Penard, J; Maugin, E; Enon, B; Picquet, J

    2007-12-01

    Peripheral vascular expressions of Coxiella burnetti Q fever are not well known. Endocarditis with negative blood culture is the most frequent clinical presentation of chronic Q fever. To date, very few cases of aneurisms or vascular grafts infections have been described. We report the case of a 54-year-old man who presented an infrarenal abdominal aorta infection, leading to a giant pseudo aneurismal formation. Blood serology and polymerase chain reaction amplification identified C. burnetti from the aortic thrombus after pseudo aneurism surgery. The treatment associated infrarenal abdominal aorta repair using a cryopreserved aorta allograft, and long-term antibiotic therapy. PMID:17851006

  18. Aortic valve endocarditis complicated by ST-elevation myocardial infarction.

    Science.gov (United States)

    Jenny, Benjamin E; Almanaseer, Yassar

    2014-12-01

    Infective endocarditis complicated by abscess formation and coronary artery compression is a rare clinical event with a high mortality rate, and diagnosis requires a heightened degree of suspicion. We present the clinical, angiographic, and echocardiographic features of a 73-year-old woman who presented with dyspnea and was found to have right coronary artery compression that was secondary to abscess formation resulting from diffuse infectious endocarditis. We discuss the patient's case and briefly review the relevant medical literature. To our knowledge, this is the first reported case of abscess formation involving a native aortic valve and the right coronary artery.

  19. Outcomes after transcatheter aortic valve implantation: transfemoral versus transapical approach

    NARCIS (Netherlands)

    Ewe, S.H.; Delgado, V.; Ng, A.C.; Antoni, M.L.; Kley, F. van der; Marsan, N.A.; Weger, A. de; Tavilla, G.; Holman, E.R.; Schalij, M.J.; Bax, J.J.

    2011-01-01

    BACKGROUND: Transcatheter aortic valve implantation is commonly implanted through a transfemoral (TFA) or transapical approach (TAA) for patients with severe aortic stenosis. This study aimed to describe the clinical and echocardiographic outcomes of TFA versus TAA. METHODS: Clinical and echocardiog

  20. [The endovascular correction of aortic coarctation].

    Science.gov (United States)

    Barkov, E D; Silin, V A; Sukhov, V K

    1992-03-01

    The endovascular balloon dilatation of the aorta coarctation performed on indications and at the optimal terms is the radical correction of the defect with a pronounced positive effect in 90% of 43 patients. No aneurysms and restenoses were found. Retained collaterals allow avoidance of the abdominal syndrome and paradoxical hypertension. Contraindications for catheter angioplasty are thought to include agenesia or considerable hypoplasia of the aorta, the absence of the aorta lumen in the zone of coarctation, its considerable length and the presence of prestenotic aneurysms. A short and noncomplicated postoperative period makes the method sufficiently economical.

  1. The German Aortic Valve Registry (GARY): in-hospital outcome

    OpenAIRE

    Hamm, Christian W.; Möllmann, Helge; Holzhey, David; Beckmann, Andreas; Veit, Christof; Figulla, Hans-Reiner; Cremer, J; Kuck, Karl-Heinz; Lange, Rüdiger; Zahn, Ralf; Sack, Stefan; Schuler, Gerhard; Walther, Thomas; Beyersdorf, Friedhelm; Böhm, Michael

    2013-01-01

    Background Aortic stenosis is a frequent valvular disease especially in elderly patients. Catheter-based valve implantation has emerged as a valuable treatment approach for these patients being either at very high risk for conventional surgery or even deemed inoperable. The German Aortic Valve Registry (GARY) provides data on conventional and catheter-based aortic procedures on an all-comers basis. Methods and results A total of 13 860 consecutive patients undergoing repair for aortic valve d...

  2. A Clinicopathological Study on Aortic Valves in Children

    Institute of Scientific and Technical Information of China (English)

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

    2007-01-01

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

  3. A patient with altered mental status and possible seizure reveals an atypical aortic dissection upon workup.

    Science.gov (United States)

    Lawal, Olufolahan J; Dhindsa, Harinder S; Loyd, Joshua W

    2014-05-01

    Aortic dissection occurs when a tear occurs in the inner muscle wall lining of the aorta, allowing blood to split the muscle layers of the aortic wall apart. It is classically characterized by pain that starts in the upper chest, which then radiates to the upper back and is tearing or ripping in quality. Our objective is to present a case followed by a brief literature review of aortic dissection and uncommon but important features that may be demonstrated. In this report, we present the case of a 57-year-old woman who was transported to the emergency department with an acute episode of altered mental status, presenting as a possible stroke with possible seizures. The patient's only complaint was mild low back pain. Physical examination revealed disorientation to time with no other neurologic deficits or abnormal findings. Results from initial noncontrast head computed tomography, chest radiograph, and laboratory studies were all normal, except for an elevated D-dimer and serum creatinine. Chest computed tomography with contrast demonstrated a type A aortic dissection. The patient was taken emergently to the operating room where the aortic valve and a portion of the ascending aorta were replaced. The patient did well and was discharged from the hospital 5 days later without any permanent sequalae. Aortic dissection is both rare and life threatening and may present with atypical signs. It is important to note that patients may show no signs of typical features or may even display other symptoms based on other branches from the aorta that have been occluded. PMID:24360026

  4. Fusobacterium necrophorum in an Abdominal Aortic Aneurysm, Treated by Once Daily Ertapenem

    OpenAIRE

    Wotherspoon, D.; Street, J. A.; Hedderwick, S.; Baker, R.

    2012-01-01

    Inflammatory aneurysms may make up a small percentage of the total number of abdominal aortic aneurysms, but they present their own unique challenges. We present a case of a 65-year-old man whose aneurysm was found to be colonized by Fusobacterium necrophorum.

  5. Loss of function mutation in LOX causes thoracic aortic aneurysm and dissection in humans.

    Science.gov (United States)

    Lee, Vivian S; Halabi, Carmen M; Hoffman, Erin P; Carmichael, Nikkola; Leshchiner, Ignaty; Lian, Christine G; Bierhals, Andrew J; Vuzman, Dana; Mecham, Robert P; Frank, Natasha Y; Stitziel, Nathan O

    2016-08-01

    Thoracic aortic aneurysms and dissections (TAAD) represent a substantial cause of morbidity and mortality worldwide. Many individuals presenting with an inherited form of TAAD do not have causal mutations in the set of genes known to underlie disease. Using whole-genome sequencing in two first cousins with TAAD, we identified a missense mutation in the lysyl oxidase (LOX) gene (c.893T > G encoding p.Met298Arg) that cosegregated with disease in the family. Using clustered regularly interspaced short palindromic repeats (CRISPR)/clustered regularly interspaced short palindromic repeats-associated protein-9 nuclease (Cas9) genome engineering tools, we introduced the human mutation into the homologous position in the mouse genome, creating mice that were heterozygous and homozygous for the human allele. Mutant mice that were heterozygous for the human allele displayed disorganized ultrastructural properties of the aortic wall characterized by fragmented elastic lamellae, whereas mice homozygous for the human allele died shortly after parturition from ascending aortic aneurysm and spontaneous hemorrhage. These data suggest that a missense mutation in LOX is associated with aortic disease in humans, likely through insufficient cross-linking of elastin and collagen in the aortic wall. Mutation carriers may be predisposed to vascular diseases because of weakened vessel walls under stress conditions. LOX sequencing for clinical TAAD may identify additional mutation carriers in the future. Additional studies using our mouse model of LOX-associated TAAD have the potential to clarify the mechanism of disease and identify novel therapeutics specific to this genetic cause.

  6. Twins with progressive thoracic aortic aneurysm, recurrent dissection and ACTA2 mutation.

    Science.gov (United States)

    Ware, Stephanie M; Shikany, Amy; Landis, Benjamin J; James, Jeanne F; Hinton, Robert B

    2014-10-01

    Thoracic aortic aneurysm (TAA) is a genetically mediated disease with variable age of onset. In the pediatric age range, nonsyndromic TAA frequently has a milder course than syndromic forms of TAA, such as Marfan syndrome or Loeys-Dietz syndrome. Herein, we describe 17-year-old identical twin brothers with severe progressive TAA due to a novel de novo ACTA2 mutation. Interestingly, both boys were diagnosed at age 11 with congenital mydriasis, a recently recognized manifestation of some ACTA2 mutations due to smooth muscle dysfunction. One of the brothers presented with acute-onset lower back pain that was identified as dissection of an abdominal aortic aneurysm. Imaging of the chest at this time showed severe fusiform TAA. Cardiac imaging in his twin showed similar TAA, but no abdominal aortic aneurysm. Both brothers underwent valve-sparing aortic root replacement, but have had progressive aortic disease with recurrent dissection requiring multiple surgeries. This case emphasizes the importance of identifying physical stigmata of smooth muscle dysfunction, such as mydriasis, as potential markers for associated aortopathy and vascular diseases.

  7. Loss of function mutation in LOX causes thoracic aortic aneurysm and dissection in humans.

    Science.gov (United States)

    Lee, Vivian S; Halabi, Carmen M; Hoffman, Erin P; Carmichael, Nikkola; Leshchiner, Ignaty; Lian, Christine G; Bierhals, Andrew J; Vuzman, Dana; Mecham, Robert P; Frank, Natasha Y; Stitziel, Nathan O

    2016-08-01

    Thoracic aortic aneurysms and dissections (TAAD) represent a substantial cause of morbidity and mortality worldwide. Many individuals presenting with an inherited form of TAAD do not have causal mutations in the set of genes known to underlie disease. Using whole-genome sequencing in two first cousins with TAAD, we identified a missense mutation in the lysyl oxidase (LOX) gene (c.893T > G encoding p.Met298Arg) that cosegregated with disease in the family. Using clustered regularly interspaced short palindromic repeats (CRISPR)/clustered regularly interspaced short palindromic repeats-associated protein-9 nuclease (Cas9) genome engineering tools, we introduced the human mutation into the homologous position in the mouse genome, creating mice that were heterozygous and homozygous for the human allele. Mutant mice that were heterozygous for the human allele displayed disorganized ultrastructural properties of the aortic wall characterized by fragmented elastic lamellae, whereas mice homozygous for the human allele died shortly after parturition from ascending aortic aneurysm and spontaneous hemorrhage. These data suggest that a missense mutation in LOX is associated with aortic disease in humans, likely through insufficient cross-linking of elastin and collagen in the aortic wall. Mutation carriers may be predisposed to vascular diseases because of weakened vessel walls under stress conditions. LOX sequencing for clinical TAAD may identify additional mutation carriers in the future. Additional studies using our mouse model of LOX-associated TAAD have the potential to clarify the mechanism of disease and identify novel therapeutics specific to this genetic cause. PMID:27432961

  8. Rapid prototyping in aortic surgery.

    Science.gov (United States)

    Bangeas, Petros; Voulalas, Grigorios; Ktenidis, Kiriakos

    2016-04-01

    3D printing provides the sequential addition of material layers and, thus, the opportunity to print parts and components made of different materials with variable mechanical and physical properties. It helps us create 3D anatomical models for the better planning of surgical procedures when needed, since it can reveal any complex anatomical feature. Images of abdominal aortic aneurysms received by computed tomographic angiography were converted into 3D images using a Google SketchUp free software and saved in stereolithography format. Using a 3D printer (Makerbot), a model made of polylactic acid material (thermoplastic filament) was printed. A 3D model of an abdominal aorta aneurysm was created in 138 min, while the model was a precise copy of the aorta visualized in the computed tomographic images. The total cost (including the initial cost of the printer) reached 1303.00 euros. 3D imaging and modelling using different materials can be very useful in cases when anatomical difficulties are recognized through the computed tomographic images and a tactile approach is demanded preoperatively. In this way, major complications during abdominal aorta aneurysm management can be predicted and prevented. Furthermore, the model can be used as a mould; the development of new, more biocompatible, less antigenic and individualized can become a challenge in the future. PMID:26803324

  9. Endovascular repair of abdominal aortic aneurysms.

    Science.gov (United States)

    Arnaoutakis, Dean J; Zammert, Martin; Karthikesalingam, Alan; Belkin, Michael

    2016-09-01

    Endovascular repair of abdominal aortic aneurysms is an important technique in the vascular surgeon's armamentarium, which has created a seismic shift in the management of aortic pathology over the past two decades. In comparison to traditional open repair, the endovascular approach is associated with significantly improved perioperative morbidity and mortality. The early survival benefit of endovascular abdominal aortic aneurysm repair is sustained up to 3 years postoperatively, but longer-term life expectancy remains poor regardless of operative modality. Nonetheless, most abdominal aortic aneurysms are now repaired using endovascular stent grafts. The technology is not perfect as several postoperative complications, namely endoleak, stent-graft migration, and graft limb thrombosis, can develop and therefore lifelong imaging surveillance is required. In addition, a postoperative inflammatory response has been documented after endovascular repair of aortic aneurysms; the clinical significance of this finding has yet to be determined. Subsequently, the safety and applicability of endovascular stent grafts are likely to improve and expand with the introduction of newer-generation devices and with the simplification of fenestrated systems. PMID:27650343

  10. Challenging Friesian horse diseases : aortic rupture and megaesophagus

    NARCIS (Netherlands)

    Ploeg, M.

    2015-01-01

    Aortic rupture is quite rare in Warmblood horses and is best known as an acute and fatal rupture of the aortic root in older breeding stallions. It has now become clear that aortic rupture, which is diagnosed around an age of 4 years, is more frequent in the Friesian breed than in others. The high p

  11. Aneurysm sac enlargement after endovascular Abdominal Aortic Aneurysm repair

    NARCIS (Netherlands)

    Dingemans, Siem A; Jonker, Frederik H W; Moll, Frans L; van Herwaarden, JA

    2015-01-01

    The aim of this study is to give an overview of current knowledge regarding abdominal aortic aneurysm (AAA) growth after endovascular aortic aneurysm repair (EVAR) that could potentially lead to aortic rupture. A search on Pubmed was performed. A total of 705 articles were found after initial search

  12. Immediate post-operative responses to transcatheter aortic valve implantation

    DEFF Research Database (Denmark)

    Egerod, Ingrid; Nielsen, Susanne; Lisby, Karen H.;

    2015-01-01

    Background:Conventional treatment for patients with severe symptomatic aortic stenosis is surgical aortic valve replacement (SAVR), but transcatheter aortic valve implantation (TAVI) has become a reliable alternative in high-risk patients.Aims:The aim of our study was to describe the post...

  13. Study of Coronary Artery Disease in Single Aortic Valvular Disease

    Institute of Scientific and Technical Information of China (English)

    张斌; 杨伟民; 占亚平

    2003-01-01

    Objectives To analyze the results of coronary angiographies (GAG) in patients with single aortic valvular heart disease; To study the relationship between aortic valve diseases and coronary artery disease (CAD). Methods 105 patients with single aortic valvular heart disease before surgery underwent angiography. The data of clinical characteristics and angiographies were analyzed. Results 51 patients had symptoms of angina pectoris among 105 patients with single aortic valvular heart disease. Seven of them were confirmed coronary artery disease by angiographies. Although the incidence of angina in aortic valve stenosis group was significantly higher than that in aortic valve regurgitation, the probability of combination of CAD in aortic valve stenosis group was similar to the later. However, the probability of combination of CAD in degenerative aortic valve group was significantly higher than the groups of rheumatic, congenitally bicuspid aortic valves, and other causes (p <0.01).Conclusions Angina pectoris is not sensitive for diagnosis of CAD in single aortic valve heart disease.The probability of combination of CAD in degenerative aortic valve disease is higher than that in aortic valve disease with other causes. Coronary angiography is strongly suggested for these patients.

  14. In situ repair of a primary Brucella-infected abdominal aortic aneurysm: long-term follow-up.

    Science.gov (United States)

    Goudard, Yvain; Pierret, Charles; de La Villéon, Bruno; Mlynski, Amélie; de Kerangal, Xavier

    2013-02-01

    Infected aortic aneurysms represent 0.85 to 1.3% of aortic aneurysms. Most often, the implicated bacteria species are Salmonella sp., Staphylococcus sp. and Streptococcus sp. Brucella-related infected aortic aneurysms are very rare. Most often, they result from endocarditis or from a local septic focus. Combined treatment by antibiotics and surgery is the standard for infectious aneurysms. In the absence of formal factual data, the surgical treatment is still discussed in the literature, especially since endovascular treatments have been in full expansion. We are reporting the case of a female patient presenting with a Brucella-related infra-renal abdominal aortic aneurysm, without primitive infectious source (area) or identified endocarditis. Surgical treatment with in situ prosthetic replacement and omentoplasty in association with adapted antibiotics allowed a favorable outcome with an excellent result after an 8-year follow up.

  15. Fenestrated endovascular aortic repair for juxtarenal abdominal aortic aneurysm

    Institute of Scientific and Technical Information of China (English)

    GUO Wei; ZHANG Hong-peng; LIU Xiao-ping; JIA Xin; XIONG Jiang; MA Xiao-hui

    2013-01-01

    Background Endovascular stent-graft with fenestration can improve proximal sealing in patients with juxtarenal abdominal aortic aneurysm (JAAA).The purpose of this study was to describe our primary experience and evaluate the safety and efficacy of fenestrated device for JAAA in high-risk patients.Methods Between March 2011 and May 2012,nine male patients (mean age,(79.6±8.6) years) with asymptomatic JAAAs underwent elective deployment of the Zenith fenestrated stent-grafts at a single institution.All patients were treated in the hybrid operating room under general anesthesia.Follow-up computed tomography angiography (CTA) was routinely performed before discharge,at 3,6,and 12 months and annually thereafter.Results Procedural success was achieved in all cases.Total sixteen small fenestrations,two large fenestrations and eight scallops were used.Intra-operative complications occurred in four patients,which included one proximal type Ⅰ endoleak,two type Ⅱ endoleaks,and one renal artery dissection.The mean hospital stay was (8.9±1.4) days,mean blood loss was (360.5±46.8) ml,and mean iodinated contrast volume was (230.6±58.3) ml.The mean follow-up time was (7.6±4.2) months.The visceral graft patency was 100% until now.One patient had an increase of serum creatinine of more than 30%,but did not require dialysis.No patients died,no stent fractured,and migration were diagnosed during the follow-up.Conclusions The early results of fenestrated device for high-risk patients with complex JAAAs are satisfactory.However,long-term fenestrated graft durability and branch vessel patency remain to be determined.

  16. Iatrogenic neonatal type B aortic dissection: comprehensive MRI-based diagnosis and follow-up

    Energy Technology Data Exchange (ETDEWEB)

    Geiger, Julia; Markl, M. [University Hospital Freiburg, Department of Diagnostic Radiology and Medical Physics, Freiburg (Germany); Stiller, B.; Arnold, R. [University Hospital Freiburg, Department of Congenital Heart Disease and Pediatric Cardiology, Freiburg (Germany); Schlensak, C. [University Hospital Freiburg, Department of Cardiovascular Surgery, Freiburg (Germany)

    2011-10-15

    Neonatal aortic dissection is rare and most frequently iatrogenic. Decision making and appropriate imaging are highly challenging for pediatric cardiologists and radiologists. We present MRI and echocardiographic findings in the follow-up at 6 months of age of a boy with a conservatively treated iatrogenic neonatal aortic dissection (type B). To evaluate the morphology of the aortic arch and descending aorta, we carried out multidirectional time-resolved three-dimensional flow-analysis and contrast-enhanced MR angiography (CE-MRA). The MRI and Doppler echocardiographic results were closely comparable. Three-dimensional visualization helped assess details of blood flow acceleration and alteration caused by the dissection, and played a key role in our deciding not to treat surgically. (orig.)

  17. Management of an aorto-esophageal fistula, complicating a descending thoracic aortic aneurysm endovascularly repaired.

    Science.gov (United States)

    Georvasili, Vaia K; Bali, Christina; Peroulis, Michalis; Kouvelos, George; Avgos, Stavros; Godevenos, Dimitris; Liakakos, Theodoros; Matsagkas, Miltiadis

    2016-04-01

    Aorto-esophageal fistula (AEF) is a rare but devastating complication of thoracic aorta endovascular repair (TEVAR). We report a case of a 64-year-old male who presented with chest pain and high CRP levels 10 months after TEVAR for a 9 cm diameter descending thoracic aortic aneurysm. The diagnosis of an AEF was confirmed and the patient was treated conservatively with broad spectrum antibiotics and total parental alimentation. After control of sepsis was achieved, esophagectomy with gastric tube reconstruction was performed and an omental pedicle was used to cover the aortic wall. No intervention to the aorta was made at that time due to the potentially infected mediastinum. The patient's recovery was uneventful and 2 years postoperatively he is in good condition and lives a normal life. Esophagectomy seems to be a mandatory stage of treatment in the setting of AEF. In cases where signs of graft infection are persistent, aortic surgery might be also necessary. PMID:24838140

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

    Science.gov (United States)

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

    2013-06-01

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

  19. Effects of Atorvastatin on Warfarin-induced Aortic Medial Calcification and Systolic Blood Pressure in Rats

    Institute of Scientific and Technical Information of China (English)

    Chengyun LIU; Jingjing WAN; Qunfang YANG; Benling QI; Wen PENG; Xuelin CHEN

    2008-01-01

    Summary: The effect of atorvastatin on warfarin-induced aortic medial calcification and systolic blood pressure (SBP) of rats induced by warfarin was studied. Thirty healthy and adult rats were randomly divided into Warfarin group (n=10), Atorvastatin group (n=10) and normal control group (n=10). Caudal arterial pressure of rats was measured once a week, and 4 weeks later, aorta was obtained. Elastic fiber, collagen fiber and calcium accumulation in tunica media of cells were measured by Von Kossa staining. The results showed that warfarin treatment led to elevation of systolic blood pressure and aortic medial calcification. The chronic treatment also increased collagen, but decreased elastin in the aorta. However, the atorvastatin treatment had adverse effects. It was concluded that treatment with atorvastatin presented evidence of blood pressure lowing and calcification reducing. These data demonstrate that atorvastatin protected aortic media from warfarin-induced calcification and elevation of systolic blood pressure.

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

    Directory of Open Access Journals (Sweden)

    Fernex Lucie

    2012-08-01

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

  1. Temporary extracorporeal bypass modalities during aortic surgery.

    Science.gov (United States)

    Bassin, Levi; Bell, David

    2016-09-01

    The key to aortic surgery is protection of the brain, heart, spinal cord, and viscera. For operations involving the aortic arch, the focus is on cerebral protection, while for pathology involving the descending thoracic aorta, the focus is on spinal protection. Optimal cerebral and spinal protection requires an extensive knowledge of the operative steps and an understanding of the cardiopulmonary bypass modalities that are possible. A bloodless field is required when operating on the aorta. As a result, periods of ischemia to the central nervous system and end-organ viscera are often unavoidable. The main techniques to mitigate ischemia include hypothermia and selective perfusion of the ischemic organ in question. This chapter will first briefly review bypass modalities and then describe how they can be used for various aortic scenarios. PMID:27650344

  2. Influence of aerobic exercise training on post-exercise responses of aortic pulse pressure and augmentation pressure in postmenopausal women

    Directory of Open Access Journals (Sweden)

    Nobuhiko eAkazawa

    2015-10-01

    Full Text Available Central arterial blood pressure (BP is more predictive of future cardiovascular events than is brachial BP because it reflects the BP load imposed on the left ventricle with greater accuracy. However, little is known about the effects of exercise training on central hemodynamic response to acute exercise. The purpose of the present study was to determine the influence of an aerobic exercise regimen on the response of aortic BP after a single aerobic exercise in postmenopausal women. Nine healthy postmenopausal women (age: 61 ± 2 years participated in a 12-week aerobic exercise training regimen. Before and after the training, each subjects performed a single bout of cycling at ventilatory thresholds for 30 min. We evaluated the post-exercise aortic BP response, which was estimated via the general transfer function from applanation tonometry. After the initial pre-training aerobic exercise session, aortic BP did not change significantly: however, aortic pulse pressure and augmentation pressure were significantly attenuated after the single aerobic exercise session following the 12-week training regimen. The present study demonstrated that a regular aerobic exercise training regimen induced the post-exercise reduction of aortic pulse pressure and augmentation pressure. Regular aerobic exercise training may enhance post-exercise reduction in aortic BP.

  3. Transcatheter CoreValve valve-in-valve implantation in a stentless porcine aortic valve for severe aortic regurgitation

    OpenAIRE

    Yong, Celina M; Buchbinder, Maurice; Giacomini, John C

    2014-01-01

    Key Clinical Message We describe the first valve-in-valve Corevalve transcatheter aortic valve replacement in the St. Jude Toronto stentless porcine aortic valve in the United States, which enabled this 59-year-old patient with a history of bacterial endocarditis and aortic regurgitation to avoid heart transplant with complete resolution of his severe left ventricular dysfunction.

  4. Hypertrophic osteoarthropathy of one leg - a sign of aortic graft infection

    Energy Technology Data Exchange (ETDEWEB)

    Spruijt, S.; Krijgsman, A.A. [Department of Orthopaedic and Trauma Surgery, Elkerliek Hospital, Helmond (Netherlands); Broek, J.A.C. van den [Department of Radiology, Elkerliek Hospital, Helmond (Netherlands); Tutein Nolthenius-Puylaert, M.C.B.J.E. [Department of Pathology, Elkerliek Hospital, Helmond (Netherlands)

    1999-04-01

    We report a rare case of hypertrophic osteoarthropathy (HOA) confined to the right leg secondary to aortic graft infection. The development of HOA exclusively localized to areas distal to a vascular prosthesis may be the presenting manifestation of graft infection and a crucial diagnostic clue in the early detection of vascular graft infection. HOA is diagnosed by its characteristic radiographic and scintigraphic pattern. Most prosthetic, especially aortic, graft infections are uniformly fatal if not treated by aggressive surgical and antibiotic therapy. Recognition of this uncommon association may facilitate an early diagnosis, which usually requires immediate surgical therapy. (orig.) With 6 figs., 28 refs.

  5. Vasorelaxant effect of diterpenoid lactones from Andrographis paniculata chloroform extract on rat aortic rings

    OpenAIRE

    R.N. Sriramaneni; Omar, Ameer Z.; Salman M Ibrahim; Sadikun Amirin; Asmawi Mohd Zaini

    2010-01-01

    Background: The aim of the present study is to evaluate the possible mechanism of the vasorelaxant effect of the Andrographis paniculata chloroform extract (APCE) and diterpenoids, such as, 14-deoxyandrographolide (DA) and 14-deoxy-11, 12-didehydroandrographolide (DDA), on rat aortic rings. Methods: DA and DDA (10 μM to 40 μM) induce relaxation in the aortic rings pre-contracted with KCl (80 mM). Results: The IC 50 values are 40.47 ± 1.44 and 37.43 ± 1.41%, respectively, and this inhibition i...

  6. Surgical Treatment of a Voluminous Infrarenal Abdominal Aortic Aneurysm with Horseshoe Kidney: Tips and Tricks.

    Science.gov (United States)

    De Caridi, Giovanni; Massara, Mafalda; Greco, Michele; Mastrojeni, Claudio; Serra, Raffaele; Salomone, Ignazio; La Spada, Michele

    2015-01-01

    Horseshoe kidney is a common urology anomaly, while its association with infrarenal abdominal aortic aneurysm represents a very rare condition. Surgical approach remains controversial however, we believe that the left retroperitoneal approach should be preferred in order to avoid isthmus resection with any subsequent renal infarction, urinary tract damage and to facilitate renal arteries reimplantation, when required. We present a case of voluminous infrarenal abdominal aortic aneurysm associated with horseshoe kidney, successfully treated through a left retroperitoneal approach on the retro-renal space. PMID:26730260

  7. Delayed Surgery for Aortic Dissection after Intravenous Thrombolysis in Acute Ischemic Stroke

    Science.gov (United States)

    Choi, Nari; Yoon, Jee-Eun; Park, Byoung-Won; Chang, Won-Ho; Kim, Hyun-Jo; Lee, Kyung Bok

    2016-01-01

    We report a case of aortic dissection masquerading as acute ischemic stroke followed by intravenous thrombolysis. A 59-year-old man presented with dizziness. After examination, the patient had a seizure with bilateral Babinski signs. Soon after identifying multiple acute infarctions in both hemispheres on diffusion-weighted brain magnetic resonance (MR) imaging, tissue plasminogen activator (t-PA) was administered. Both common carotid arteries were invisible on MR angiography, and subsequent chest computed tomography revealed an aortic dissection. The emergency operation was delayed for 13 hours due to t-PA administration. The patient died of massive bleeding. PMID:27734002

  8. Aortoduodenal fistula following aortic reconstruction of a pseudoaneurysm caused by stab wound 12 years ago

    Institute of Scientific and Technical Information of China (English)

    Jian-cang ZHOU; Qiu-ping XU; Lai-gen SHEN; Kong-han PAN; Yi-ping MOU

    2009-01-01

    Gastrointestinal bleeding due to aortoenteric fistula is extremely rare. Aortoenteric fistula is difficult to be diagnosed timely and entails a significant morbidity and mortality. Herein, we present an uncommon case of gastrointestinal bleeding caused by aortoduodenal fistula, which was a complication of a successful aortic reconstruction 4 months ago for an aortic pseudoaneurysm resulted from a stab wound 12 years ago. An urgent laparotomy confirmed an aortoduodenal fistula and repaired the defects in aorta and duodenum, but a prolonged shock led to the patient's death. In summary, early diagnosis and surgical intervention for aortoenteric fistula are vital for survival.

  9. De novo periaortic fibrosis after endovascular aortic repair.

    Science.gov (United States)

    Alomran, Faris; de Blic, Romain; Mallios, Alexandros; Costanzo, Alessandro; Boura, Benoit; Combes, Myriam

    2014-02-01

    We report a case of a 63-year-old man presenting with abdominal pain and hydronephrosis secondary to periaortic fibrosis (PAF) 8 months after an endovascular aortic repair (EVAR) using a woven polyester bifurcated graft. De novo delayed PAF after open repair is rare and even more infrequent after EVAR. All 3 previously reported cases occurred after woven polyester grafts and no reported cases after polytetrafluorethylene grafts. Management included steroidal anti-inflammatory treatment and bilateral double J tube placement. Satisfactory results were obtained. PMID:24275428

  10. Multimodality Imaging of a Giant Aortic Valve Papillary Fibroelastoma

    Directory of Open Access Journals (Sweden)

    Nowell M. Fine

    2013-01-01

    Full Text Available Papillary fibroelastomas (PFEs are benign cardiac tumors arising from endocardium. They are commonly found on valvular surfaces and average 1.0–1.5 cm in size. Though often asymptomatic, PFEs can lead to potentially severe complications, primarily due to their embolic potential. Surgical resection is recommended for all symptomatic or large PFEs. We report the case of a patient presenting with cardiovascular symptoms who was found to have a very large aortic valve PFE, as diagnosed by histopathologic examination following surgical resection. Multimodality cardiovascular imaging demonstrates the classic morphologic findings, including a pedunculated appearance and oscillating “frond-like” surface projections.

  11. An uncommon cause of chest pain - penetrating atherosclerotic aortic ulcer

    OpenAIRE

    Kyaw, Htoo; Sadiq, Sanah; Chowdhury, Arnab; Gholamrezaee, Rashin; Yoe, Linus

    2016-01-01

    Chest pain is a very common symptom and can be of cardiac or non-cardiac origin. It accounts for approximately 5.5 million annual emergency room visits in the United States, according to 2011 CDC data. Penetrating atherosclerotic aortic ulcer (PAU), an uncommon condition, is also a potential cause of chest pain. We here report the case of a 65-year-old woman who presented with atypical chest and back pain. The pain persisted for 4 weeks necessitating two emergency room visits. Initial tests w...

  12. Infectious or Noninfectious? Ruptured, Thrombosed Inflammatory Aortic Aneurysm with Spondylolysis

    Energy Technology Data Exchange (ETDEWEB)

    Stefanczyk, Ludomir; Elgalal, Marcin, E-mail: telgalal@yahoo.co.uk [Medical University of Lodz, Department of Radiology and Diagnostic Imaging (Poland); Papiewski, Andrzej [Medical University of Lodz, Department of Gastroenterological Surgery (Poland); Szubert, Wojciech [Medical University of Lodz, Department of Radiology and Diagnostic Imaging (Poland); Szopinski, Piotr [Institute of Hematology and Transfusion Medicine, Clinic of Vascular Surgery (Poland)

    2013-06-15

    Osteolysis of vertebrae due to inflammatory aortic aneurysm is rarely observed. However, it is estimated that up to 10 % of infectious aneurysms coexist with bone tissue destruction, most commonly the vertebrae. Inflammatory aneurysms with no identified infection factor, along with infiltration of adjacent muscle and in particular extensive destruction of bone tissue have rarely been described in the literature. A case of inflammatory aneurysm with posterior wall rupture and inflammatory infiltration of the iliopsoas muscle and spine, together with extensive vertebral body destruction, is presented. The aneurysm was successfully treated with endovascular aneurysm repair EVAR.

  13. Infective Endocarditis and Aortic Valve Abscess in an Infant.

    Science.gov (United States)

    Williamson, Kristy A; Gmuca, Sabrina; Rosman, Eliyahu C; Thomas, Philomena

    2015-09-01

    Infective endocarditis is relatively uncommon in the pediatric population, but when it does occur, results in substantial morbidity and mortality. Children at risk for endocarditis are typically those with an underlying congenital heart condition. Furthermore, an endocardial abscess is a very rare yet serious complication of infective endocarditis. We describe a case of a 23-month-old previously healthy male infant with no known congenital heart disease who returned to the emergency department after a recent hospitalization for pneumococcal bacteremia, presenting acutely ill but without fever. He was found to be in congestive heart failure due to endocarditis and an aortic root abscess.

  14. Anesthesia Approach in Endovascular Aortic Reconstruction

    Directory of Open Access Journals (Sweden)

    Ayşin Alagöl

    2013-03-01

    Full Text Available Introduction: We have analyzed our initial results of our anesthesia techniques in our new-onset endovascular aortic reconstruction cases.Patients and Methods: The perioperative data of 15 elective and emergent endovascular aortic reconstruction cases that were operated in 2010-2011 were collected in a database. The choice of anesthesia was made by the risk factors, surgical team’s preferences, type and location of the aortic pathology and by the predicted operation duration. The data of local and general anesthesia cases were compared.Results: Thirteen (86.7% cases were male and 2 (13.3% female. Eleven patients were in ASA Class III. The demographic parameters, ASA classifications, concurrent diseases were similar in both groups. Thirteen (86.7% cases had infrarenal abdominal aortic aneurysm and 2 (13.3% had Type III aortic dissection. The diastolic arterial pressures were lower in general anesthesia group in 20th and 40th minutes’ measurements just like the mean arterial pressure measurements at the 40th, 100th minutes and during the deployment of the graft. Postoperative mortality occurred in 3 (20.0% patients and they all had general anesthesia and they were operated on emergency basis. Postoperative morbidity occurred in four patients that had general anesthesia (acute renal failure, multi-organ failure and pneumonia. The other patient had atrial fibrillation on the 1st postoperative day and was converted to sinus rhythm with amiodarone infusion.Conclusion: Edovascular aortic reconstruction procedures can safely be performed with both general and local anesthesia less invasively compared to open surgery. General anesthesia may be preferred for the better hemodynamic control.

  15. Chylous complications after abdominal aortic surgery.

    Science.gov (United States)

    Haug, E S; Saether, O D; Odegaard, A; Johnsen, G; Myhre, H O

    1998-12-01

    Two patients developed chylous complications following abdominal aortic aneurysm repair. One patient had chylous ascitis and was successfully treated by a peritoneo-caval shunt. The other patient developed a lymph cyst, which gradually resorbed after puncture. Chylous complications following aortic surgery are rare. Patients in bad a general condition should be treated by initial paracentesis and total parenteral nutrition, supplemented by medium-chain triglyceride and low-fat diet. If no improvement is observed on this regimen, the next step should be implementation of a peritoneo-venous shunt, whereas direct ligation of the leak should be reserved for those who are not responding to this treatment. PMID:10204656

  16. Contemporary management of blunt aortic trauma.

    Science.gov (United States)

    Dubose, J J; Azizzadeh, A; Estrera, A L; Safi, H J

    2015-10-01

    Blunt thoracic aortic injury (BTAI) remains a common cause of death following blunt mechanisms of trauma. Among patients who survive to reach hospital care, significant advances in diagnosis and treatment afford previously unattainable survival. The Society for Vascular Surgery (SVS) guidelines provide current best-evidence suggestions for treatment of BTAI. However, several key areas of controversy regarding optimal BTAI care remain. These include the refinement of selection criteria, timing for treatment and the need for long-term follow-up data. In addition, the advent of the Aortic Trauma Foundation (ATF) represents an important development in collaborative research in this field. PMID:25868973

  17. Aortic valve replacement in octogenarians

    Directory of Open Access Journals (Sweden)

    Dark John H

    2007-07-01

    Full Text Available Abstract Background and Aims As our population ages and life expectancy increases the number of people aged over 80 and more referred for cardiac surgery is growing. This study sought to identify the outcome of aortic valve replacement (AVR in octogenarians. Methods 68 patients aged 80 years or more underwent AVR at the Freeman Hospital, between April 2001 and April 2004. A retrospective review of the notes and outcomes from the patients' GP and the NHS strategic tracking service was performed. 54% (37 underwent isolated AVR whilst 46% (31 underwent combined AVR and CABG. Results Follow up was 100% complete. The mean age was 83.1 ± s.d. 2.9 years, a mean gradient of 83 ± s.d. 31 mmHg and mean AVA of 0.56 cm2. The mean additive EuroSCORE was 8.6 ± s.d. 1.2, the logistic EuroSCORE mean 12.0 ± s.d. 5.9. In hospital 30 day mortality was 13 %. Survival was 80% at 1 year and 78% at 2 years. Median follow up was for 712 days. Stepwise logistic regression identified chronic obstructive airways disease as an independent predictor of mortality (p Conclusion Our study demonstrates that the operative mortality for AVR in the over eighties is good, whilst the mid to long term outcome is excellent There is a very low attrition rate with those undergoing the procedure living as long than their age matched population. This study confirms AVR is a safe, acceptable treatment for octogenarians with excellent mid term outcomes.

  18. Aortic Dissection and Renal Failure in a Patient with Severe Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Valerie Brooke

    2012-01-01

    Full Text Available Acute aortic dissection (AAD is a life-threatening condition associated with high morbidity and mortality. The most important recognized acquired cause that leads to dissection is chronic arterial hypertension. With respect to the anuria and renal failure, aortic dissection is not something that is always considered and is still not a very common presentation unless both renal arteries come off the false lumen of the dissection. However, when present, preoperative renal failure in patients with acute type B dissection has been noted to be an independent predictor of mortality. Early recognition and diagnosis is the key and as noted by previous studies as well, almost a third of these patients are initially worked up for other causes until later when they are diagnosed with aortic dissection. Here we present a case of a patient presenting with severe hypothyroidism, long-standing hypertension, and anuria. Through the case, we highlight the importance of having aortic dissection as an important differential in patients presenting with anuria who have a long standing history of uncontrolled hypertension. Pathophysiology relating to severe hypothyroidism-induced renal dysfunction is also discussed.

  19. Congenital quadricuspid aortic valve: analysis of 11 surgical cases

    Institute of Scientific and Technical Information of China (English)

    TANG Yang-feng; XU Ji-bin; HAN Lin; LU Fang-lin; LANG Xi-long; SONG Zhi-gang; XU Zhi-yun

    2011-01-01

    Background Congenital quadricuspid aortic valve is rarely seen during aortic valve replacement (AVR).The diagnosis and treatment of the disease were reported in 11 cases.Methods Eleven patients (nine men and two women,mean age 33.4 years) with quadricuspid aortic valve were retrospectively evaluated.Medical records,echocardiograms and surgical treatment were reviewed.Results In accordance with the Hurwitz and Roberts classification,the patients were classified as type A (n=2),type B (n=7),type F (n=1) and type G (n=1).Three patients were associated with other heart diseases,including infective endocarditis and mitral prolaps,left superior vena cava,aortic aneurysm.All had aortic regurgitation (AR) except two with aortic stenosis (AS),detected by color-flow Doppler echocardiography.The congenital quadricuspid aortic valve deformity in seven patients was diagnosed by echocardiography.All patients underwent successful aortic valve replacement.Conclusion Quadricuspid aortic valve is a rare cause of aortic insufficiency,while echocardiography plays an important role in diagnosing the disease.Aortic valve replacement is the major therapy for the disease.

  20. Experimental study on aortic remodeling in sinoaortic denervated rats

    Institute of Scientific and Technical Information of China (English)

    MIAO Chao-yu; TAO Xia; GUAN Yun-feng; YANG You-cai; CHU Zheng-xu; SU Ding-feng

    2001-01-01

    Objective: To study the aortic remodeling produced by chronic sinoaortic denervation (SAD) and its time course, and to study the role of humoral factor in the SAD-induced aortic remodeling. Methods: In rats with chronic SAD or sham operation, the aortic structure was measured by computer-assisted image analysis, the aortic function by isolated artery preparation, and angiotensin Ⅱ concentration by radioimmunoassay. Results and Conclusion: The aortic structural remodeling developed progressively at 4, 8, 16 and 32 weeks after SAD. Aortic structural remodeling after SAD expressed mainly as aortic hypertrophy due to SMC growth and collagen accumulation. The aortic contraction elicited by norepinephrine (NE) was progressively increased 8, 16 and 32 weeks after SAD. The aortic relaxation elicited by acetylcholine (ACh) was depressed 8, 16 and 32 weeks after SAD. In addition, in 32-week SAD rats the NE-induced contraction was not increased by endothelial denudation. These indicated that the increased contraction and depressed relaxation after SAD were related to the change of endothelium and/or the change of interaction between endothelium and SMC. In 10-week SAD rats, plasma angiotensin Ⅱ concentration remained unchanged, whereas aortic angiotensin Ⅱ concentration was significantly increased, suggesting that activation of tissue renin-angiotensin system may be involved in SAD-induced aortic remodeling.