WorldWideScience

Sample records for aortic hypoplasia presented

  1. Diagnosis of abdominal aortic hypoplasia by state-of-the-art MR angiography

    International Nuclear Information System (INIS)

    Abdominal aortic hypoplasia is a rare vascular variant with possible major clinical sequelae when the renal arteries are also involved. The condition is thought to result from embryonic overfusion of the two dorsal aortas. The diagnosis may be considered in patients presenting with hypertension in the neonatal period and severely reduced or absent arterial pulses in the groin. We present contrast-enhanced state-of-the-art magnetic resonance angiography imaging findings in an 8-year-old boy with abdominal aortic hypoplasia. (orig.)

  2. Brainstem hypoplasia presenting with mirror movement

    Directory of Open Access Journals (Sweden)

    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.

  3. Multiple myeloma presenting with coexisting severe marrow hypoplasia.

    Science.gov (United States)

    Medhi, K; Kalita, Dipti; Chopra, Anita; Anand, Mona; Raina, Vinod; Kumar, Rajive

    2008-01-01

    A 68-year-old man was referred to us with clinical and bone marrow (BM) features compatible with aplastic anemia. The correct diagnosis, hypoplasia of the BM coexisting with multiple myeloma, became apparent after noting rouleaux in the peripheral blood (PB) and approximately 50% plasma cells in the touch imprint of one of the two BM biopsies done. As standard therapy was precluded, the patient was put on dexamethasone but died within 4 days. This first case of the coexistence of untreated myeloma with aplastic BM shows that even apparently straightforward hypoplasia seen on the BM biopsy should be interpreted in conjunction with the PB smear and the BM touch imprint findings. Among other things, the BM biopsy and imprint should be repeated if the PB has findings such as rouleaux that do not fit with straightforward aplastic anemia. The combination of myeloma and BM aplasia precludes standard therapy and is rapidly fatal. PMID:19008591

  4. Multiple myeloma presenting with coexisting severe marrow hypoplasia

    Directory of Open Access Journals (Sweden)

    Medhi K

    2008-10-01

    Full Text Available A 68-year-old man was referred to us with clinical and bone marrow (BM features compatible with aplastic anemia. The correct diagnosis, hypoplasia of the BM coexisting with multiple myeloma, became apparent after noting rouleaux in the peripheral blood (PB and approximately 50% plasma cells in the touch imprint of one of the two BM biopsies done. As standard therapy was precluded, the patient was put on dexamethasone but died within 4 days. This first case of the coexistence of untreated myeloma with aplastic BM shows that even apparently straightforward hypoplasia seen on the BM biopsy should be interpreted in conjunction with the PB smear and the BM touch imprint findings. Among other things, the BM biopsy and imprint should be repeated if the PB has findings such as rouleaux that do not fit with straightforward aplastic anemia. The combination of myeloma and BM aplasia precludes standard therapy and is rapidly fatal.

  5. Multiple myeloma presenting with coexisting severe marrow hypoplasia

    OpenAIRE

    Medhi K; Kalita Dipti; Chopra Anita; Anand Mona; Raina Vinod; Kumar Rajive

    2008-01-01

    A 68-year-old man was referred to us with clinical and bone marrow (BM) features compatible with aplastic anemia. The correct diagnosis, hypoplasia of the BM coexisting with multiple myeloma, became apparent after noting rouleaux in the peripheral blood (PB) and approximately 50% plasma cells in the touch imprint of one of the two BM biopsies done. As standard therapy was precluded, the patient was put on dexamethasone but died within 4 days. This first case of the coexistence of untre...

  6. 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

  7. Unilateral pulmonary hypoplasia

    Directory of Open Access Journals (Sweden)

    Albay S

    2008-10-01

    Full Text Available Pulmonary hypoplasia represents a broad range of malformations characterized by incomplete development of lung tissue. The severity of the lesion depends on the appearance time of the malformation during the timeline of lung development, and the presence of further anatomic anomalies. In this report, we present a case of pulmonary hypoplasia in a 27-year old woman.

  8. Cerebellar Hypoplasia

    Science.gov (United States)

    ... 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 ...

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

    OpenAIRE

    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 ...

  10. Takayasu arteritis with dilated cardiomyopathy and aortic dissection: a rare presentation

    International Nuclear Information System (INIS)

    We report a peculiar case of TA in a young male Pakistani patient presenting with dilated cardiomyopathy (DCM) and aortic dissection. To our knowledge this is the first reported case of TA with DCM and aortic dissection simultaneously. (author)

  11. 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...

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

    OpenAIRE

    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...

  13. Rare presentation of ruptured syphilitic aortic aneurysm with pseudoaneurysm.

    Science.gov (United States)

    de Almeida Feitosa, Israel Nilton; Dantas Leite Figueiredo, Magda; de Sousa Belem, Lucia; Evelin Soares Filho, Antônio Wilon

    2015-11-01

    We report the interesting case of a rare form of presentation of rupture of the ascending aorta with formation of a pseudoaneurysm, diagnosed following the development of a large mass on the surface of the chest over a period of about eight months. Serological tests were positive for syphilis. Echocardiography and computed tomography angiography were essential to confirm the diagnosis and therapeutic management. Cardiovascular syphilis is a rare entity since the discovery of penicillin. Rupture of an aortic aneurysm with formation of a pseudoaneurysm is a potentially fatal complication. The postoperative period was uneventful and the patient was discharged from hospital within days of surgery. PMID:26481180

  14. Biliary hypoplasia in Williams syndrome

    OpenAIRE

    O'Reilly, K; Ahmed, S F; Murday, V.; McGrogan, P

    2006-01-01

    Neonatal hepatitis and biliary hypoplasia are not recognised features of Williams syndrome. A case of Williams syndrome, presenting with neonatal conjugated hyperbilirubinaemia leading to an initial misdiagnosis is reported.

  15. Hybrid Treatment of Acute Abdominal Aortic Thrombosis Presenting with Paraplegia.

    Science.gov (United States)

    Azzarone, Matteo; De Troia, Alessandro; Iazzolino, Luigi; Nabulsi, Bilal; Tecchio, Tiziano

    2016-05-01

    Acute thrombotic or embolic occlusion of the abdominal aorta is a rare vascular emergency associated with high morbidity and mortality rates. Classically, the clinical presentation is a severe peripheral ischemia with bilateral leg pain as the predominant feature. Aortic occlusion presenting as an isolated acute onset of paraplegia due to spinal cord ischemia is very rare and requires improved awareness to prevent adverse outcomes associated with delayed diagnosis. We report the case of a 54-year-old man who presented with sudden paraplegia due to the thrombotic occlusion of the infrarenal aorta involving the first segment of the common iliac arteries on both sides; emergent transperitoneal aorto iliac thrombectomy combined with the endovascular iliac kissing-stent technique were performed achieving perioperative complete regression of the symptoms. PMID:26968371

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

    International Nuclear Information System (INIS)

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1984-02-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    OpenAIRE

    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 ...

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

    Institute of Scientific and Technical Information of China (English)

    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.

  1. Aortic dissection presenting as acute subtotal left main coronary artery occlusion: a case approach and review of the literature.

    Science.gov (United States)

    Ruisi, Michael; Fallahi, Arzhang; Lala, Moinakhtar; Kanei, Yumiko

    2015-05-01

    Aortic dissection is the most common fatal condition of the aorta, yet it is often missed on initial clinical presentation. Aortic dissection associated with acute coronary syndrome (ACS) is relatively rare, but if it occurs, it can be diagnostically challenging, and the condition can be fatal. Here we describe a case of aortic dissection presenting as ST-segment elevation myocardial infarction (STEMI) managed via the transradial approach. We describe the current literature on the subject. PMID:25780485

  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

    Directory of Open Access Journals (Sweden)

    Sharon H. Gnagi

    2015-01-01

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

  4. Syphilitic Aortic Aneurysm in a Young HIV-Infected Man: Case Presentation.

    Science.gov (United States)

    Cataño, Juan Carlos; Ramirez, Isabel Cristina

    2011-01-01

    We describe the case of a young HIV-positive man who presented to the emergency room in hypovolemic shock. During subsequent evaluation, we documented a huge aortic aneurysm consistent with tertiary syphilis. The final autopsy demonstrated the extent of cardiovascular compromise caused by this aneurysm. PMID:22567481

  5. MR findings in pontocerebellar hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    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

    Directory of Open Access Journals (Sweden)

    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.

    Science.gov (United States)

    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. Vermian aplasia and hypoplasia

    International Nuclear Information System (INIS)

    This paper reports vermian aplasia or hypoplasia unrelated to Dandy-Walker complex or Joubert syndrome. Vermian hypoplasia is most commonly associated with the malformations of Dandy-Walker cyst or variant. There have been few reports of familial vermian anomaly (Joubert syndrome) with episodes of polypnea and apnea. Five patients with vermian hypoplasia or absence were prospectively discovered among 1,130 children undergoing brain MR imaging. Ages ranged from 2 mo to 15 y at the time of discovery: The abnormalities were poorly defined in three patients who had previously undergone CT. There was no common link in these patients with a variable number of other systemic, brain, or facial abnormalities. No unifying symptoms were identified. Two patients had the MR imaging appearance of Joubert syndrome, but none had the cyclical respiratory symptoms usually associated with this syndrome

  9. 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; Weigang, Ernst; Livi, Ugolino; Verzini, Fabio; Schmidli, Jürg; Chiesa, Roberto; Melissano, Germano; Kahlberg, Andrea; Amabile, Philippe; Harringer, Wolfgang; Horacek, Michael; Erbel, Raimund; Park, Kay-Hyun; Beyersdorf, Friedhelm; Rylski, Bartosz; Blanke, Philipp; Canaud, Ludovic; Khoynezhad, Ali; Lönn, Lars; Rousseau, Hervè; Trimarchi, Santi; Brunkwall, Jan; Gawenda, Michael; Dong, Zhihui; Fu, Weiguo; Schuster, Ingrid; Grimm, Michael

    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)....

  10. [Evaluation after 20 years of a case of Takayasu's disease that presented with aortic regurgitation].

    Science.gov (United States)

    Pierre, B; Machet, M C; Marchand, M; Alison, D; Casset-Senon, D; Fauchier, L; Charniot, J C; Babuty, D; Cosnay, P

    2005-03-01

    Takayasu's disease is a segmental multifocal affection of medium and large arteries. The diagnosis is based on the association of stenotic and aneurismal lesions of the aorta and its branches secondary to an inflammatory infiltration of the media and adventitia. Cases of aortic regurgitation associated with aneurismal dilatation of the ascending aorta as the presenting features of Takayasu's disease, as in this case, are rare. Histological examination of the aortic wall may help establish the diagnosis by showing signs of aortitis. The other usual arterial lesions are sometimes missing at the initial phase of the disease. A late histological diagnosis may be difficult as the inflammatory lesions tend to be progressively replaced by fibrotic lesions or a banal atheroma. PMID:15816330

  11. 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.

  12. 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

  13. Hemifacial microsomia with pulmonary hypoplasia

    OpenAIRE

    Panigrahi, Inusha; Das, Rashmi Ranjan; Marwaha, Ram Kumar

    2010-01-01

    Hemifacial microsomia (HFM) is the second most common facial anomaly after cleft lip and palate. HFM is characterised by facial asymmetry, microtia, preauricular tags, macrostomia and cardiac defects. The majority of cases are sporadic, although autosomal dominant and recessive modes of inheritance have been reported. Here, the case of an 11-month-old boy with HFM and pulmonary hypoplasia, which is a rare association, is described. Pulmonary hypoplasia was detected during investigation of the...

  14. 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

  15. 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

  16. Imaging a boa constrictor--the incomplete double aortic arch syndrome.

    Science.gov (United States)

    Narayan, Rajeev L; Kanwar, Anubhav; Jacobi, Adam; Sanz, Javier

    2012-11-01

    Incomplete double aortic arch is a rare anomaly resulting from atresia rather than complete involution in the distal left arch resulting in a non-patent fibrous cord between the left arch and descending thoracic aorta. This anatomic anomaly may cause symptomatic vascular rings, leading to stridor, wheezing, or dysphagia, requiring surgical transection of the fibrous cord. Herein, we describe an asymptomatic 59 year-old man presenting for contrast-enhanced CT angiography to assess cardiac anatomy prior to radiofrequency ablation, who was incidentally found to have an incomplete double aortic arch with hypoplasia of the left arch segment and an aortic diverticulum. Recognition of this abnormality by imaging is important to inform both corrective surgery in symptomatic patients, as well as assist in the planning of percutaneous coronary and vascular interventions. PMID:22542042

  17. Percutaneous closure of an aortic prosthetic paravalvular leak with device in a patient presenting with heart failure

    Directory of Open Access Journals (Sweden)

    Altug Osken

    2015-01-01

    Full Text Available Paravalvular leaks (PVLs are a well-recognized complication of prosthetic valve replacement. Perivalvular prosthetic regurgitation causes significant morbidity and is associated with high perioperative mortality if open surgical repair is required. PVLs manifest with symptoms of congestive heart failure, hemolysis, or in most cases, the combination of both. In recent years, the development of imaging and device techniques significantly shortened the duration of fluoroscopy and procedure success was achieved. Percutaneous transcatheter closure of PVLs with a specific device causes symptomatic improvement. We present a case of transcatheter closure of aortic paravalvular insufficiency with amplatzer duct occluder 2 device.

  18. 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

  19. Aortic stenosis

    Science.gov (United States)

    ... stenosis; Valvular aortic stenosis; Congenital heart - aortic stenosis; Rheumatic fever - aortic stenosis Images Aortic stenosis Heart valves References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil ...

  20. 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: ...

  1. 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.

  2. 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...

  3. 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.

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

    OpenAIRE

    Yu, Hui-Chun; Wang, Zhen-Qing; Hao, Yuan-Yuan; An, Feng-Ping; Hu, Yu-Chuan; Deng, Rui-Bing; Yu, Peng; Cui, Guang-Bin; Li, He

    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 angiography showed acute thoracic aortic dissection, DeBakey type IIIb, a dissecting aneurysm in the proximal descending thoracic aorta starting immediately after the origin of the left subclavian artery and extending distally below the re...

  5. 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

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

    Science.gov (United States)

    Yu, Hui-Chun; Wang, Zhen-Qing; Hao, Yuan-Yuan; An, Feng-Ping; Hu, Yu-Chuan; Deng, Rui-Bing; Yu, Peng; Cui, Guang-Bin; Li, He

    2015-05-01

    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 angiography showed acute thoracic aortic dissection, DeBakey type IIIb, a dissecting aneurysm in the proximal descending thoracic aorta starting 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. PMID:26089858

  7. Unusual Anatomic Variations Associated With Bilateral Ulnar Artery Hypoplasia.

    Science.gov (United States)

    Ro, Hyung-Suk; Roh, Si-Gyun; Shin, Jin Yong; Lee, Nae-Ho; Yang, Kyung-Moo

    2016-05-01

    Variations and anomalies of upper extremities have been commonly reported in routine dissection, clinical practices, and cadaver studies. Despite ongoing research on arterial variations of upper extremities, the absence of bilateral ulnar artery is extremely rare with only 3 patients reported. As the authors are presenting a successfully treated patient, initially prepped for radial forearm osteocutaneous free flap for treatment on oromandibular defect after a wide resection of head and neck cancer lesion, being confirmed to have bilateral ulnar artery hypoplasia and due to this, the patient had to change her surgical plan to fibular osteocutaneous free flap. PMID:27100648

  8. 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

  9. Bilateral hind limb hypoplasia in a foal

    International Nuclear Information System (INIS)

    Hypoplasia affecting both hind limbs was observed in a neonatal foal. Scintigraphy aided in establishing diagnosis and prognosis. Radiography revealed an abnormally shaped middle phalanx and lack of the distalphalanx and navicular bone in the right hind limb, with an abnormally shaped distal phalanx and navicular bone in the left hind limb. Scintigraphic findings were closely correlated with the radiographic findings. Radiographic and scintigraphic findings were confirmed at postmortem examination

  10. 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.

  11. Rate of enamel formation and hypoplasia timing

    OpenAIRE

    Al-Shorman, Abdulla; Alrousan, Mohammad; Khwaileh, Ali

    2014-01-01

    Linear Enamel Hypoplasia (LEH) is a decrease in enamel thickness resulting from a temporary disruption in enamel formation during times of dental development. To establish a statistical relationship between the timing of enamel formation and the length of the crown, the published data of Reid and Dean (2006) were statistically analyzed using SPSS. The results indicate that the relationship between the crown length and the days of enamel formation is nonlinear as proposed by previous studies b...

  12. Soft palate hypoplasia in a horse

    International Nuclear Information System (INIS)

    Several imaging techniques were used to diagnose hypoplasia of the soft palate in a horse. The absence of the caudal soft palate, hypertrophied lymphoid tissue and the formation of a pseudouvula were observed endoscopically. Plain and contrast radiography were used to demonstrate a soft palate remnant and to identify structures rostral to the epiglottis. Retrograde endoscopy of the pharynx via a tracheotomy incision is described

  13. Causes of congenital unilateral pulmonary hypoplasia

    International Nuclear Information System (INIS)

    A review of the roentgenograms and clinical records of 33 children with primary congenital underdevelopment of one lung showed that 9 patients had simple pulmonary hypoplasia, 8 had anomalous venous return to the right atrium or the inferior vena cava (scimitar syndrome), 7 had an absence of ipsilateral pulmonary artery, 7 had an accessory diaphragm, and 2 had a pulmonary sequestration adjacent to a small diaphragmatic hernia. (orig.)

  14. 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

  15. 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

    2015-01-01

    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. PMID:26464410

  16. 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

  17. 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.)

  18. Spectral domain optical coherence tomography and microperimetry in foveal hypoplasia

    Directory of Open Access Journals (Sweden)

    Swakshyar Saumya Pal

    2011-01-01

    Full Text Available A case of foveal hypoplasia associated with ocular albinism with anatomic and functional changes by various techniques using spectral domain optical coherence tomography (SD-OCT, microperimeter and confocal scanning laser ophthalmoscope is described. This case highlights the importance of microperimeter in detecting the functional abnormalities of vision and SD-OCT in identifying the retinal laminar abnormalities in foveal hypoplasia.

  19. 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

  20. 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

  1. 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

  2. Brain stem hypoplasia associated with Cri-du-Chat syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Jin Ho; Lee, Ha Young; Lim, Myung Kwan; Kim, Mi Young; Kang, Young Hye; Lee, Kyung Hee; Cho, Soon Gu [Dept. of Radiology, Inha University Hospital, Inha University School of Medicine, Incheon (Korea, Republic of)

    2013-12-15

    Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

  3. Brain stem hypoplasia associated with Cri-du-Chat syndrome

    International Nuclear Information System (INIS)

    Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

  4. Treatment of severe maxillary cleft hypoplasia in a case with missing premaxilla with anterior maxillary distraction using tooth-borne hyrax appliance

    Directory of Open Access Journals (Sweden)

    Akshai Shetty

    2015-01-01

    Full Text Available Cleft orthodontics generally poses a challenge and a missing premaxilla adds to the difficulty in managing them. The lack of bone support and anterior teeth in a case with missing premaxilla accounts not only for difficulty in rehabilitation but also in increasing the maxillary hypoplasia. This article presents a case report where planned orthodontic and surgical management using distraction has helped treat a severe maxillary hypoplasia in a patient with missing premaxilla. The treatment plan and method can be used to treat severe maxillary hypoplasia and yield reasonably acceptable results for such patients.

  5. Aortic dissection: case series

    Directory of Open Access Journals (Sweden)

    Bhavana Venkata Nagabhushana Rao

    2016-04-01

    Full Text Available Aortic dissection may not be attended by a physician in his lifetime, but he should possess all the clinical acumen to deal with as it is a catastrophic disease. Early and accurate diagnosis will save a life. Here we present three cases we faced in sequence over a period of two months. A case of extensive dissection arch to thoracic aorta, its display in detail. Second case eliciting ambiguity between coronary ischemia and aortic dissection. Management difficulties of such clinician situation are discussed. Third case, the fracture of a renal artery stent leading to severe hypertension, abdominal pain, and aortic dissection. Such case was not described in the literature to our knowledge. [Int J Res Med Sci 2016; 4(4.000: 1268-1271

  6. Hydatid cyst involving the aortic arch.

    Science.gov (United States)

    Apaydin, Anil Z; Oguz, Emrah; Zoghi, Mehdi

    2007-03-01

    We report a very rare case of primary mediastinal hydatid cyst which invaded the ascending aorta and the aortic arch which initially presented as a cranial mass. Aortic wall is a very unusual site for the hydatid cysts. To the best of our knowledge, this is the first reported case of hydatid cyst located within the aortic arch lumen. Patient underwent ascending aortic and hemiarch replacement under hypothermic circulatory arrest and removal of the cyst. Patient had an uneventful recovery and has been on follow-up. Although the literature data are very limited, we believe that the aortic procedure of choice should be graft interpositon rather than patch repair. PMID:17215134

  7. 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

  8. 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

  9. 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

  10. Combination esthetic treatment of anterior teeth affected by idiopathic enamel hypoplasia: a case report.

    Science.gov (United States)

    Roberts, Donald L; Warner, Ben F; Bentley, Dan A; Quock, Ryan L

    2016-01-01

    The esthetic management of enamel dysplasia presents an opportunity for the practitioner to integrate expertise in diagnosis, biomaterials, and technique. In addition to considerations of appearance, careful attention must be paid to occlusal function and restorative material properties. This case report highlights a combined approach of conservative lithium disilicate indirect restorations and composite resin direct veneers to electively restore the anterior teeth of a patient diagnosed with idiopathic enamel hypoplasia. PMID:27148656

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

    OpenAIRE

    Masuda N; Hasegawa T; Yamashita M; Ogata N

    2014-01-01

    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...

  12. Association between enamel hypoplasia and dental caries in primary second molars and permanent first molars: A 3-year follow-up study

    OpenAIRE

    Sakeenabi Basha; Roshan Noor Mohamed; Hiremath Shivalinga Swamy

    2016-01-01

    Context: Enamel hypoplasia is a defect caused by disturbances during enamel formation. These defects in the enamel present important clinical significance as they predispose a tooth to dental caries. Aim: The aim of this study was to assess the longitudinal relationships between enamel hypoplasia and caries experience of primary second molars and permanent first molars. Materials and Methods: The study sample consisted of 765 subjects who underwent dental examinations at both ages 6 and 9 by ...

  13. 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

  14. 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

  15. 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.

  16. 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.

  17. Recurrent tamponade and aortic dissection in syphilis.

    Science.gov (United States)

    Stansal, Audrey; Mirault, Tristan; Rossi, Aude; Dupin, Nicolas; Bruneval, Patrick; Bel, Alain; Azarine, Arshid; Minozzi, Catherine; Deman, Anne Laure; Messas, Emmanuel

    2013-11-01

    Syphilitic cardiovascular disease has been described since the 19th century, mainly on autopsy series. Major clinical manifestations are aortic aneurysm, aortic insufficiency, and coronary ostial stenosis. The diagnosis of syphilitic cardiovascular disease is based mainly on positive serologic tests and overt clinical manifestations. We present here a rare and unusual clinical presentation of a tertiary syphilis with recurrent tamponade and type B aortic dissection, whose positive diagnosis was made by polymerase chain reaction on pericardial fluid analysis. PMID:24182507

  18. MDCT evaluation of acute aortic syndrome (AAS).

    Science.gov (United States)

    Valente, Tullio; Rossi, Giovanni; Lassandro, Francesco; Rea, Gaetano; Marino, Maurizio; Muto, Maurizio; Molino, Antonio; Scaglione, Mariano

    2016-05-01

    Non-traumatic acute thoracic aortic syndromes (AAS) describe a spectrum of life-threatening aortic pathologies with significant implications on diagnosis, therapy and management. There is a common pathway for the various manifestations of AAS that eventually leads to a breakdown of the aortic intima and media. Improvements in biology and health policy and diffusion of technology into the community resulted in an associated decrease in mortality and morbidity related to aortic therapeutic interventions. Hybrid procedures, branched and fenestrated endografts, and percutaneous aortic valves have emerged as potent and viable alternatives to traditional surgeries. In this context, current state-of-the art multidetector CT (MDCT) is actually the gold standard in the emergency setting because of its intrinsic diagnostic value. Management of acute aortic disease has changed with the increasing realization that endovascular therapies may offer distinct advantages in these situations. This article provides a summary of AAS, focusing especially on the MDCT technique, typical and atypical findings and common pitfalls of AAS, as well as recent concepts regarding the subtypes of AAS, consisting of aortic dissection, intramural haematoma, penetrating atherosclerotic ulcer and unstable aortic aneurysm or contained aortic rupture. MDCT findings will be related to pathophysiology, timing and management options to achieve a definite and timely diagnostic and therapeutic definition. In the present article, we review the aetiology, pathophysiology, clinical presentation, outcomes and therapeutic approaches to acute aortic syndromes. PMID:27033344

  19. 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

  20. Unilateral segmental odontomaxillary hypoplasia: an unusual case report

    Energy Technology Data Exchange (ETDEWEB)

    Pandey, Sushma; Pai, Keerthilatha M; Nayak, Ajay G; Vineetha, Ravindranath [Manipal College of Dental Sciences, Manipal (India)

    2011-03-15

    Facial asymmetry is not an uncommon occurrence in day to day dental practice. It can be caused by various etiologic factors ranging from facial trauma to serious hereditary conditions. Here, we report a rare case of non-syndromic facial asymmetry in a young female, who was born with this condition but was not aware of the progression of asymmetry. No relevant family history was recognized. She was also deficient in both deciduous and permanent teeth in the corresponding region of maxilla. Hence, the cause of this asymmetry was believed to be a segmental odontomaxillary hypoplasia of left maxilla accompanied by agenesis of left maxillary premolars and molars and disuse atrophy of corresponding facial musculature. This report briefly discussed the comparative features of segmental odontomaxillary hypoplasia, hemimaxillofacial dysplasia, and segmental odontomaxillary dysplasia and justified the differences between segmental odontomaxillary hypoplasia and the other two conditions.

  1. 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)

  2. 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...

  3. Aortic valve bypass

    DEFF Research Database (Denmark)

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

    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...

  4. 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

  5. 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

  6. 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...

  7. Inherited neuroaxonal dystrophy in dogs causing lethal, fetal-onset motor system dysfunction and cerebellar hypoplasia

    OpenAIRE

    Fyfe, John C.; Al-Tamimi, Raba' A.; Castellani, Rudy J.; Rosenstein, Diana; Goldowitz, Daniel; Henthorn, Paula S.

    2010-01-01

    Neuroaxonal dystrophy in brainstem, spinal cord tracts, and spinal nerves accompanied by cerebellar hypoplasia was observed in a colony of laboratory dogs. Fetal akinesia was documented by ultrasonographic examination. At birth, affected puppies exhibited stereotypical positioning of limbs, scoliosis, arthrogryposis, pulmonary hypoplasia, and respiratory failure. Regional hypoplasia in the central nervous system was apparent grossly, most strikingly as underdeveloped cerebellum and spinal cor...

  8. Two novel prenatal treatment strategies for experimental left ventricular hypoplasia.

    Czech Academy of Sciences Publication Activity Database

    Sedmera, David; Dealmeida, A.; McQuinn, T. C.

    Vienna: ESC, 2007, s. 103-104. [Annual Congress of the European Society of Cardiology 2007. Vienna (AT), 01.09.2007-05.09.2007] Institutional research plan: CEZ:AV0Z50450515 Keywords : hypoplasia Subject RIV: FA - Cardiovascular Diseases incl. Cardiotharic Surgery

  9. 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.

  10. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... the primary reason that we end up offering patients aortic valve surgery in our community. Aortic stenosis ... a picture of the aortic valve from a patient who had a very diseased aortic valve. And ...

  11. 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...

  12. 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.

  13. Bovine aortic arch: A novel association with thoracic aortic dilation

    International Nuclear Information System (INIS)

    Aim: To investigate whether there is a link between bovine arch and thoracic aortic aneurysm. Materials and methods: Computed tomography (CT) and magnetic resonance imaging (MRI) images of the thorax of 191 patients with dilated thoracic aortas and 391 consecutive, unselected patients as controls were retrospectively reviewed. Bovine arch was considered present if either a shared origin of the left common carotid and innominate arteries or an origin of the left common carotid from the innominate artery was identified. A chi-square test was used to evaluate the significance of differences between subgroups. Results: A trend towards increased prevalence of bovine arch was seen in patients with dilated aortas (26.2%) compared to controls (20.5%, p = 0.12). The association was statistically significant in patients over 70 years old (31.9%, p = 0.019) and when dilation involved the aortic arch (47.6%, p = 0.003). Conclusions: An association between bovine arch and aortic dilation is seen in older patients, and when dilation involves the aortic arch. Bovine arch should be considered a potential risk factor for thoracic aortic aneurysm.

  14. 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 ...

  15. 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 ...

  16. 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

  17. 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.

  18. Aortic growth rates in chronic aortic dissection

    International Nuclear Information System (INIS)

    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)

  19. 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...

  20. Aesthetic approach for anterior teeth with enamel hypoplasia

    OpenAIRE

    Josué Martos; Andréa Gewehr; Emanuele Paim

    2012-01-01

    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 cons...

  1. Optic nerve hypoplasia and astigmatism: a new association.

    OpenAIRE

    Zeki, S. M.

    1990-01-01

    Thirty-one patients with optic nerve hypoplasia (ONH) or septo-optic dysplasia and two patients with segmental ONH underwent retinoscopy. The results were compared with those of 20 normal subjects. There was a higher prevalence of astigmatism in the patients than in the controls. Two patients with segmental ONH had no evidence of astigmatism. The association of astigmatism with ONH has not hitherto been reported. The close association of ONH with astigmatism highlights the importance of perfo...

  2. Classification, diagnosis and potential mechanisms in Pontocerebellar Hypoplasia

    Directory of Open Access Journals (Sweden)

    Poll-The Bwee

    2011-07-01

    Full Text Available Abstract 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/atrophy of cerebellum and pons, progressive microcephaly, and variable cerebral involvement. Patients have severe cognitive and motor handicaps and seizures are often reported. Treatment is only symptomatic and prognosis is poor, as most patients die during infancy or childhood. The genetic basis of different subtypes has been elucidated, which makes prenatal testing possible in families with mutations. Mutations in three tRNA splicing endonuclease subunit genes were found to be responsible for PCH2, PCH4 and PCH5. Mutations in the nuclear encoded mitochondrial arginyl- tRNA synthetase gene underlie PCH6. The tRNA splicing endonuclease, the mitochondrial arginyl- tRNA synthetase and the vaccinia related kinase1 are mutated in the minority of PCH1 cases. These genes are involved in essential processes in protein synthesis in general and tRNA processing in particular. In this review we describe the neuroradiological, neuropathological, clinical and genetic features of the different PCH subtypes and we report on in vitro and in vivo studies on the tRNA splicing endonuclease and mitochondrial arginyl-tRNA synthetase and discuss their relation to pontocerebellar hypoplasia.

  3. 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

  4. Multimodality Imaging Approach towards Primary Aortic Sarcomas Arising after Endovascular Abdominal Aortic Aneurysm Repair: Case Series Report.

    Science.gov (United States)

    Kamran, Mudassar; Fowler, Kathryn J; Mellnick, Vincent M; Sicard, Gregorio A; Narra, Vamsi R

    2016-06-01

    Primary aortic neoplasms are rare. Aortic sarcoma arising after endovascular aneurysm repair (EVAR) is a scarce subset of primary aortic malignancies, reports of which are infrequent in the published literature. The diagnosis of aortic sarcoma is challenging due to its non-specific clinical presentation, and the prognosis is poor due to delayed diagnosis, rapid proliferation, and propensity for metastasis. Post-EVAR, aortic sarcomas may mimic other more common aortic processes on surveillance imaging. Radiologists are rarely knowledgeable about this rare entity for which multimodality imaging and awareness are invaluable in early diagnosis. A series of three pathologically confirmed cases are presented to display the multimodality imaging features and clinical presentations of aortic sarcoma arising after EVAR. PMID:26721588

  5. Stroke Volume estimation using aortic pressure measurements and aortic cross sectional area: Proof of concept.

    Science.gov (United States)

    Kamoi, S; Pretty, C G; Chiew, Y S; Pironet, A; Davidson, S; Desaive, T; Shaw, G M; Chase, J G

    2015-08-01

    Accurate Stroke Volume (SV) monitoring is essential for patient with cardiovascular dysfunction patients. However, direct SV measurements are not clinically feasible due to the highly invasive nature of measurement devices. Current devices for indirect monitoring of SV are shown to be inaccurate during sudden hemodynamic changes. This paper presents a novel SV estimation using readily available aortic pressure measurements and aortic cross sectional area, using data from a porcine experiment where medical interventions such as fluid replacement, dobutamine infusions, and recruitment maneuvers induced SV changes in a pig with circulatory shock. Measurement of left ventricular volume, proximal aortic pressure, and descending aortic pressure waveforms were made simultaneously during the experiment. From measured data, proximal aortic pressure was separated into reservoir and excess pressures. Beat-to-beat aortic characteristic impedance values were calculated using both aortic pressure measurements and an estimate of the aortic cross sectional area. SV was estimated using the calculated aortic characteristic impedance and excess component of the proximal aorta. The median difference between directly measured SV and estimated SV was -1.4ml with 95% limit of agreement +/- 6.6ml. This method demonstrates that SV can be accurately captured beat-to-beat during sudden changes in hemodynamic state. This novel SV estimation could enable improved cardiac and circulatory treatment in the critical care environment by titrating treatment to the effect on SV. PMID:26736434

  6. Genitopatellar syndrome: a new condition comprising absent patellae, scrotal hypoplasia, renal anomalies, facial dysmorphism, and mental retardation

    OpenAIRE

    Cormier-Daire, V.; Chauvet, M.; Lyonnet, S; Briard, M.; Munnich, A; Le Merrer, M

    2000-01-01

    We report on the association of absent patellae, genital and renal anomalies, dysmorphic features, and mental retardation in seven children (six boys and one girl) belonging to five unrelated families. Flexion deformities of the knees and hips with club feet and absent patellae were consistently observed and scrotal hypoplasia and cryptorchidism were present in all boys (6/6). Dysmorphic features included a coarse face, a large nose with a high nasal bridge, and microcephaly. Other features i...

  7. 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

  8. Complete graft dehiscence 8 months after repair of acute type A aortic dissection

    OpenAIRE

    Gebhard, Cathérine; Biaggi, Patric; Stähli, Barbara E; Schwarz, Urs; Felix, Christian; Falk, Volkmar

    2013-01-01

    Acute type A aortic dissection is a dreaded differential diagnosis of acute chest pain. Long-term outcome mainly depends on pre-existing comorbidities and post-operative complications. We present a patient with aortic graft dehiscence and subsequent severe aortic regurgitation due to fungal graft infection 8 months after repair of acute type A aortic dissection. Redo aortic surgery had to be delayed for 28 days due to intracerebral haemorrhage caused by septic embolism and clipping of a mycot...

  9. 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.

  10. 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.

  11. 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.

  12. 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

  13. CT diagnosis of aortic graft infections

    International Nuclear Information System (INIS)

    Two cases of aortic graft infections diagnosed by computed tomography (CT) are presented. CT scans demonstrated a zone of low attenuation in the perigraft area, in one case with a rim enhancement. The diagnoses were verfied by operation. (orig.)

  14. 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.)

  15. 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 ... to progression and aortic sclerosis is not a reason to need an operation or aggressive management at ...

  16. 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 ...

  17. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... surgery in our community. Aortic stenosis is a process by which the aortic valve, which is the ... and does not open easily or appropriately. This process starts out as a disease we call aortic ...

  18. 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.

  19. 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

  20. 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 ...

  1. 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.

  2. 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...

  3. 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.)

  4. 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.)

  5. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available Aortic Aneurysm Repair May 7, 2009 Good afternoon. Welcome to the Baptist Cardiac and Vascular Institute here in Miami. My name ... our live webcast. Today we’re going to repair an abdominal aortic aneurysm using a technology called ...

  6. 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 ...

  7. 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

  8. A rare cause of recurrent aortic dissection

    Directory of Open Access Journals (Sweden)

    Yashwant Agrawal

    2016-07-01

    Full Text Available 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.

  9. Presentations

    International Nuclear Information System (INIS)

    The presented materials consist of presentations of international workshop which held in Warsaw from 4 to 5 October 2007. Main subject of the meeting was progress in manufacturing as well as research program development for neutron detector which is planned to be placed at GANIL laboratory and will be used in nuclear spectroscopy research

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

    Science.gov (United States)

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

    2013-01-01

    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. PMID:24964430

  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. 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.

  14. 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)

  15. Optic nerve hypoplasia in fetal alcohol syndrome: an update.

    Science.gov (United States)

    Pinazo-Duran, M D; Renau-Piqueras, J; Guerri, C; Strömland, K

    1997-01-01

    Optic nerve hypoplasia was detected in up to one half of a group of Swedish children born to alcoholic mothers. Using an experimental model of pre- and postnatal alcohol exposure in rats fed a liquid diet, reduced optic nerve size from gestational day 21 (294 +/- 26 x 10(2) microns2 vs 502 +/- 16 x 10(2) microns2; n = 6; p alcohol levels achieved in dams and their offspring. Altered glial cells and degenerating and atrophic optic axons, myelin sheaths and ganglion cells were frequent in the alcohol-exposed optic nerves. Smaller optic nerve (1.918 +/- 61 x 10(2) microns2 vs 2.195 +/- 40 x 10(2) microns2; n = 4; p alcohol exposure. In summary, alcohol as a major teratogenic agent may induce dysmorphogenesis and irremediable damage to the retina and optic nerve, which frequently manifests itself as hypoplastic optic nerve. PMID:9352281

  16. 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

  17. 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 ...

  18. [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

  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. Presentations

    International Nuclear Information System (INIS)

    The PARIS meeting held in Cracow, Poland from 14 to 15 May 2007. The main subjects discussed during this meeting were the status of international project dedicated to gamma spectroscopy research. The scientific research program includes investigations of giant dipole resonance, probe of hot nuclei induced in heavy reactions, Jacobi shape transitions, isospin mixing and nuclear multifragmentation. The mentioned programme needs Rand D development such as new scintillations materials as lanthanum chlorides and bromides as well as new photo detection sensors as avalanche photodiodes - such subjects are also subjects of discussion. Additionally results of computerized simulations of scintillation detectors properties by means of GEANT- 4 code are presented

  1. Presentation

    OpenAIRE

    Eduardo Vicente; Rosana de Lima Soares; Eduardo Victorio Morettin

    2013-01-01

    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 ...

  2. 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

  3. 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).

  4. 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

  5. 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.

  6. 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.

  7. 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

  8. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... Rua, and together as a team of multidisciplinary physicians, we’re going to repair an abdominal aortic ... takes a special type of training. Both the doctors in the room are board certified and highly ...

  9. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... the age of 65. It’s most common in males. There is an increasing number, due to the ... The risk factors for abdominal aortic aneurysms are males over 60, hardening of the arteries, which is ...

  10. 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 ...

  11. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... this procedure. So let’s go back now and learn a little bit about abdominal aortic aneurysms. Great. ... And one of the things that I’m learning from this movie as we looking at these ...

  12. 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 ...

  13. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... to the aging baby boomers. Next slide. The risk factors for abdominal aortic aneurysms are males over 60, ... doing a good examination and also accessing for risk factors. So we have a very integrated team here, ...

  14. 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 ...

  15. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... this is to prevent rupture and to prevent death from rupture. This area just underneath the renal ... Okay. Abdominal aortic aneurysms cause approximately 15,000 deaths in the United States each year. It affects ...

  16. 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

  17. 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 countr...... southern European countries. Imbalances in the prevalence of rheumatic heart disease, health resource availability and variations in surgical practice throughout Europe might be possible etiological causes....

  18. 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.)

  19. Hypoplasia of bone marrow and cytopenia in non-hemic diseases

    International Nuclear Information System (INIS)

    Common (stereotype) and different links in pathogenesis of hemodepressions pronounced in the form of cytopenia and/or hypoplasia of hemopoiesis are considered. Pathogenesis and signs of the diseases, as well as their diagnosis, are studied in detail

  20. 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 ...

  1. Left ventricular outflow tract false aneurysm late after aortic valve replacement.

    Science.gov (United States)

    Bizzarri, Federico; Braconi, Lucio; Rossi, Alessandra; Sorbara, Carlo; Stefano, Pier Luigi

    2005-01-01

    We describe an unusual case of left ventricular outflow tract (LVOT) pseudoaneurysm late after aortic valve replacement. A 77-year-old man, who had undergone aortic valve replacement with mechanical prosthesis 7 years ago, presented, asymptomatic, with a transesophageal echocardiography (TTE) diagnosis of a large cavitary mass arising behind the aortic wall. The orifice of the pseudoaneurysm was successfully surgically closed and the aortic root reconstructed with cryopreserved homograft. PMID:15870043

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

    Directory of Open Access Journals (Sweden)

    Sarah Farrell

    2010-08-01

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

  3. 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...

  4. Circular Enamel Hypoplasia: A Rare Enamel Developmental Disturbance in Permanent Teeth

    OpenAIRE

    Sandhu, Meera; Gulia, Shweta; Nagpal, Mehak; Sachdev, Vinod

    2014-01-01

    Circular enamel hypoplasia, a type of enamel hypoplasia is an extensive enamel disturbance that results in a demarcating line surrounding the crown of the injured teeth visible both clinically and radiographically that most frequently occurs as a result of trauma in children around the age of two years. Clinical features include poor aesthetics, dentin sensitivity, increased susceptibility to dental caries and malocclusion. Early radiographic diagnosis of such teeth is important for timely in...

  5. Association between Enamel Hypoplasia and Dental Caries in Primary Second Molars: A Cohort Study

    OpenAIRE

    Hong, L.; Levy, S.M.; Warren, J.J.; Broffitt, B.

    2009-01-01

    The purpose of this study was to assess the longitudinal relationships between enamel hypoplasia and caries experience of primary second molars. The study sample was 491 subjects who received dental examinations at both age 5 and 9 by the calibrated examiners. Four primary second molars (n = 1,892) were scored for the presence of enamel hypoplasia for each participant. Caries presence and number of decayed and filled surfaces (dfs) were determined at age 5 and 9. The relationships between ena...

  6. Are Aortic Stent Grafts Safe in Pregnancy?

    OpenAIRE

    Nader Khandanpour; Mehta, Tapan A.; Adiseshiah, M; Meyer, Felicity J.

    2015-01-01

    Aortic stent grafts are increasingly used to treat aortic aneurysms and also other aortic pathologies. The safety of aortic stent grafts in pregnancy has never been studied or reported. We report on two cases of aortic stent grafts in pregnant women and discuss the effect of pregnancy on these aortic stent grafts.

  7. 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

  8. 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...

  9. 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

  10. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... aortic stenosis, are there any activities that I should avoid doing? That's a great question, Jim. Generally ... do not have symptoms but have aortic stenosis should be considered for surgery. Age, in and of ...

  11. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... who have symptomatic aortic stenosis of a severe nature. It's even been liberalized in some patient populations ... the heart. The aortic valve, because of its nature being in back of the heart, is not ...

  12. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... up offering patients aortic valve surgery in our community. Aortic stenosis is a process by which the ... basic valve types that we use in our practice, those being tissue valves and mechanical valves. Tissue ...

  13. 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 ...

  14. 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 ...

  15. 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 ...

  16. 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 ...

  17. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... see aortic stenosis in patients younger, in their 40s and 50s, if they have congenitally bicuspid, or ... a year and, you know, probably 30 to 40 minimally invasive aortic valve replacements a year. So ...

  18. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... Hospital in Pinehurst, North Carolina. During the program, it's easy for you to learn about the procedure. ... the aortic valve, and proceeds to aortic stenosis. It's really not felt to be due to wear ...

  19. 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

  20. 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

  1. Supravalvular aortic stenosis with sudden cardiac death

    Directory of Open Access Journals (Sweden)

    Pradeep Vaideeswar

    2015-01-01

    Full Text Available Sudden cardiac death (SCD most commonly results from previously undiagnosed congenital, acquired, or hereditary cardiac diseases. Congenital aortic valvular, subvalvular, and supravalvular disease with left ventricular outflow tract obstruction is an important preventable cause of sudden death. This report documents sudden death presumably due to acute myocardial ischemia in a young male with an undiagnosed supravalvular aortic stenosis (SVAS due to a rare association of isolation of coronary sinuses of Valsalva. Congenital supravalvular pulmonary stenosis and mitral valvular dysplasia were also present.

  2. 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...

  3. Abdominal aortic aneurysm demonstrated on renal scintigraphy.

    Science.gov (United States)

    Phisitkul, Sorot; Brian, Susan; Rakvit, Ariwan; Jenkins, Leigh A; Bohannon, W Todd; Harris, Jennifer; Tsikouris, James; Silva, Michael B; Meyerrose, Gary E

    2003-08-01

    A 74-year-old hypertensive woman presented with abdominal discomfort and a pulsatile abdominal mass. Anterior abdominal angiography during cardiac blood pool, and renal scintigraphic imaging demonstrated a large abdominal aortic aneurysm. 1, 2 Before endovascular repair with an aortoiliac endograft, the abdominal aneurysm measured 7.5 x 7.0 cm on abdominal computed tomography. This study demonstrates that a suspected abdominal aortic aneurysm can be confirmed using the addition of anterior abdominal imaging with normal posterior imaging at the time of renal scintigraphy. PMID:12897671

  4. EXTRAPULMONARY SEQUESTRATION WITH PULMONARY HYPOPLASIA AND MULTICYSTIC RENAL DYSPLASIA : A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Anita

    2015-05-01

    Full Text Available Pulmonary sequestration is a rare anomaly which consists of the presence of pulmonary tissue that is not attached to the rest of the lung and does not communicate with the trachea. [1] It could be intrapulmonary or extrapulmonary. We report a case of extrapulmonary sequestration with brief review of literature. A 22 years old primigravida underwent an ultrasonography at 24 weeks of gestation which revealed a single live fetus with bilateral pleural effusion, fetal hydrops and the fetal thorax showed mediastinal shift to the right. A hyperechoic mass was present in the left thoracic cavity with a systemic blood supply to it. Termination of pregnancy was advised as the findings were incompatible with life and the fetus autopsied. Significant gross findings were a hypoplastic left lung, a grey - white spongy mass adjacent to the left lung but no t attached to it and present outside the pleural cavity which derived its blood supply via a branch from the thoracic aorta and caused a shift in the mediastinal structures to the right. Both kidneys showed multiple cystic spaces. Microscopically the mass showed multiple cystically dilated alveolar spaces and ducts lined by cuboidal to tall columnar epithelium, the left lung showed features of pulmonary hypoplasia and the microscopic findings in both the kidneys were suggestive of multicystic renal dysplasi a. Hence, it was reported as a case of left sided extrapulmonary sequestration with hypoplastic left lung and bilateral renal cystic dysplasia.

  5. 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.

  6. 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)

  7. Natural history of abdominal aortic aneurysm

    DEFF Research Database (Denmark)

    Perko, M J; Schroeder, T V; Olsen, P S;

    1993-01-01

    During a 10-year period in which 735 patients presented with abdominal aortic aneurysms to our clinic, 63 were not offered operative treatment. The primary reason for choosing conservative treatment was concomitant diseases that increased the risk of operation. After 2 years of followup, half of ...

  8. 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 ...

  9. 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 ...

  10. 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 ...

  11. 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.

  12. Bilateral ostial coronary stenosis and rheumatic aortic valve stenosis.

    Science.gov (United States)

    Sorokin, Alexeyi; Weich, Hellmuth; Doubell, Anton; Moolman, Johannes A

    2006-01-01

    A 49-year-old patient presented with angina pectoris and clinical findings of aortic valve stenosis and regurgitation. Rheumatic aortic valve stenosis and regurgitation was diagnosed on echocardiography. Coronary angiography findings showed severe calcification in the aorta root with right coronary ostial occlusion, and were suggestive of left main ostial stenosis and proximal main stem stenosis, which was confirmed on CT angiography. Curvilinear calcification of the aorta was present on CT angiography. The findings suggested syphilitic aortitis. Syphilis serology was positive (RPR titre 1/16). The angina was caused by severe coronary ostial disease likely due to syphilitic aortitis and exacerbated by the rheumatic aortic valve stenosis and regurgitation. PMID:16885079

  13. Aortic valve annuloplasty: new single suture technique.

    Science.gov (United States)

    Schöllhorn, Joachim; Rylski, Bartosz; Beyersdorf, Friedhelm

    2014-06-01

    Reconstruction strategies for aortic valve insufficiency in the presence of aortic annulus dilatation are usually surgically challenging. We demonstrate a simple, modified Taylor technique of downsizing and stabilization of the aortic annulus using a single internal base suture. Since April 2011, 22 consecutive patients have undergone safe aortic valve annuloplasty. No reoperations for aortic valve insufficiency and no deaths occurred. PMID:24882316

  14. 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

  15. 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.)

  16. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    International Nuclear Information System (INIS)

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)

  17. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Ilkko, E.; Tikkakoski, T.; Pyhtinen, J. [Department of Radiology, Oulu University Hospital, Kajaanintie 50, 90220 Oulu (Finland)

    1998-11-01

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)

  18. 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 ...

  19. 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

  20. Endovascular aortic repair: first twenty years.

    Science.gov (United States)

    Koncar, Igor; Tolić, Momcilo; Ilić, Nikola; Cvetković, Slobodan; Dragas, Marko; Cinara, Ilijas; Kostić, Dusan; Davidović, Lazar

    2012-01-01

    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. PMID:23350259

  1. 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

  2. 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

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

    Science.gov (United States)

    Katiyar, Sarika; Ganjsinghani, Payal Kamlesh; Jain, Rajnish Kumar

    2015-01-01

    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 PMID:26379295

  4. Imaging of thoracic aortic dissection

    International Nuclear Information System (INIS)

    Acute thoracic aortic dissection has a high mortality rate if untreated, so the diagnosis must be rapidly made. Multiple imaging techniques are often used. This retrospective study from 1988 to 1993 assesses the usefulness in diagnosis of chest X-rays, computed tomography (CT) scanning, aortography, magnetic resonance imaging (MRI), trans-thoracic (TTE) and trans-oesophageal (TOE) echocardiography. Forty-two patients with a final clinical diagnosis of dissection were studied. The diagnosis was confirmed in 16 (13 at surgery and three at autopsy). Three died with dissection given as the only cause of death. Chest X-ray abnormalities were seen in all 19 patients with surgery or death from dissection, with a widened mediastinum and/or dilated aorta being present in 17. In the group of 16 patients with surgery or autopsy proof, CT scans found dissections in 9 out of 12 patients studied and correctly classified the type in only five. Aortography was preformed in five, with accurate depiction of dissection and type in all. TTE found dissections in three of eight patients imaged by this method. MRI and TOE were preformed each on two patients, with accurate depiction of dissection and type in each. Because of the relatively low sensitivity of CT scanning in defining aortic dissections Westmead Hospital is currently assessing the use of TOE as the prime imaging modality prior to surgical intervention. 17 refs., 4 tabs., 4 figs

  5. Aortic Aneurysm: A Rare Cause of Ortner's Syndrome

    International Nuclear Information System (INIS)

    A young man presented with hoarseness of voice and was found to have left vocal cord paralysis and a large opacity on chest X-ray in the left upper zone. CT angiography showed a giant aneurysm of the aortic arch involving the left subclavian artery. Using a dual perfusion system, with the femoral bypass circuit taking care of the spinal protection and the aortic bypass circuit providing the cerebral protection, the aneurysm was excised and a 16 mm Dacron graft was anastomosed to the aortic arch and the left subclavian artery was anastomosed to the interposition graft. He had a smooth postoperative course and his hoarseness subsided in next 6 months. (author)

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

    OpenAIRE

    Strahm Carol; Lederer Heidi; Schwarz Esther I; Bachli Esther B

    2012-01-01

    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. ...

  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. Thoracic aortic aneurysm and dissection.

    Science.gov (United States)

    Goldfinger, Judith Z; Halperin, Jonathan L; Marin, Michael L; Stewart, Allan S; Eagle, Kim A; Fuster, Valentin

    2014-10-21

    Aortic dissection is the most devastating complication of thoracic aortic disease. In the more than 250 years since thoracic aortic dissection was first described, much has been learned about diseases of the thoracic aorta. In this review, we describe normal thoracic aortic size; risk factors for dissection, including genetic and inflammatory conditions; the underpinnings of genetic diseases associated with aneurysm and dissection, including Marfan syndrome and the role of transforming growth factor beta signaling; data on the role for medical therapies in aneurysmal disease, including beta-blockers, angiotensin receptor blockers, and angiotensin-converting enzyme inhibitors; prophylactic surgery for aneurysm; surgical techniques for the aortic root; and surgical and endovascular management of aneurysm and dissection for different aortic segments. PMID:25323262

  9. Abdominal aortic aneurysm surgery

    DEFF Research Database (Denmark)

    Gefke, K; Schroeder, T V; Thisted, B; Olsen, P S; Perko, M J; Agerskov, Kim; Røder, O; Lorentzen, Jørgen Ewald

    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 55......, 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.......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...

  10. Blunt Traumatic Aortic Injury of Right Aortic Arch in a Patient with an Aberrant Left Subclavian Artery

    Science.gov (United States)

    Yeo, Daryl Li-Tian; Haider, Sajjad; Zhen, Claire Alexandra Chew

    2015-01-01

    Right-sided aortic arch (RAA) is a rare congenital developmental variant present in about 0.1 percent of the population. This anatomical anomaly is commonly associated with congenital heart disease and complications from compression of mediastinal structures. However, it is unknown if patients are at a higher risk of blunt thoracic aortic injury (BTAI). We report a case of a 20-year-old man admitted to the hospital after being hit by an automobile. Computed tomographic scan revealed an RAA with an aberrant left subclavian artery originating from a Kommerell’s diverticulum. A pseudo-aneurysm was also seen along the aortic arch. A diagnosis of blunt traumatic aortic injury was made. The patient was successfully treated with a 26mm Vascutek hybrid stentgraft using the frozen elephant trunk technique. A literature review of the pathophysiology of BTAI was performed to investigate if patients with right-sided aortic arch are at a higher risk of suffering from BTAI. Results from the review suggest that although theoretically there may be a higher risk of BTAI in RAA patients, the rarity of this condition has prevented large studies to be conducted. Previously reported cases of BTAI in RAA have highlighted the possibility that the aortic isthmus may be anatomically weak and therefore prone to injury. We have explored this possibility by reviewing current literature of the embryological origins of the aortic arch and descending aorta. PMID:25745378

  11. Breast hypoplasia following irradiation of the female breast in infancy and early childhood

    International Nuclear Information System (INIS)

    The occurrence and the degree of breast hypoplasia were studied in 129 women irradiated with ionizing radiation in infancy or childhood for hemangioma located in the breast region. The patients were born between 1934 and 1943 and were treated at Radiumhemmet before 4 years of age. Dosimetry was based on treatment data. The mean absorbed dose to the breast anlage was 2.3 Gy. Breast asymmetry was estimated by a mailed questionnaire to all patients and by a clinical examination of 53 patients living in Stockholm Country. Breast hypoplasia on the treated side was reported by 57% of the patients and on the contralateral side by 8%. A breast hypoplasia exceeding 10% was found in 53% of the clinically examined patients on the treated side and in 9% on the contralateral side. The frequency and the severity of impaired breast development increased with the absorbed dose. The possibility of a threshold dose for the occurrence of breast hypoplasia could neither be established nor ruled out. (orig.)

  12. 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.

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

    International Nuclear Information System (INIS)

    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.)

  14. 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.

  15. 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

  16. Acute aortic intramural hematoma

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Oh Keun; Choi, Yo Won; Kim, Kwon Hyung; Jeon, Seok Chol; Park, Choong Kee; Seo, Heung Suk; Hahm, Chang Kok [Hanyang Univ. College of Medicine, Seoul (Korea, Republic of)

    1997-02-01

    To evaluate the radiologic findings of acute intramural hematoma of the aorta, and the clinical follow up thereof. Among 34 cases confirmed clinically and radiologically as aortic dissection, and analysis was carried out based on 15 cases in which intramural hematoma without false lumen was demonstrated, on initial CT, 12 cases of in which follow up CT was used and five cases involving an aortogram. Elements such as the shape of the thickened aortic wall, ulcer-like intimal defects, and intimal calcification were examined. Changes in these elements were also examined on follow-up CT. DeBackey types 1 and 3 accounted for one and 14 cases, respectively. Initial precontrast CT demonstrated continuous, crescentic high attenuation areas along the wall of the descending aorta. In postcontrast scans, the crescentic areas were of relatively lower-attenuation and appeared along the aorta wall. Displaced intimal calcifications were seen in nine of fifteen patients. There was no intimal flap on all five aortogram, while aortic wall thickening and atherosclerotic change were demonstrated in four cases and in one case, respectively. Focal ulcers were seen in three cases. Ulcer-like intimal defects were demonstrated in a total of eleven cases (eight on CT, two on aortogram, and one on both). In ten of the twelve cases seen on follow up CT, the thickness of the intramural hematoma was seen to be reduced. Among the 15 cases, the operation was performed in two cases, and the remaining 13 received conservative treatment. In ten cases observed for more than twelve months, a recurrence of symptoms did not occur. Eccentric aortic wall thickening in patients who complain of acute chest pain is the result of acute aortic dissection with intramural hematoma, or a penetrating atherosclerotic ulcer of the aorta. The later may be differentiated from the former by the presence of on ulcer-like intimal defect. When both diseases are limited to the descending aorta, conservative treatment may

  17. Acute aortic intramural hematoma

    International Nuclear Information System (INIS)

    To evaluate the radiologic findings of acute intramural hematoma of the aorta, and the clinical follow up thereof. Among 34 cases confirmed clinically and radiologically as aortic dissection, and analysis was carried out based on 15 cases in which intramural hematoma without false lumen was demonstrated, on initial CT, 12 cases of in which follow up CT was used and five cases involving an aortogram. Elements such as the shape of the thickened aortic wall, ulcer-like intimal defects, and intimal calcification were examined. Changes in these elements were also examined on follow-up CT. DeBackey types 1 and 3 accounted for one and 14 cases, respectively. Initial precontrast CT demonstrated continuous, crescentic high attenuation areas along the wall of the descending aorta. In postcontrast scans, the crescentic areas were of relatively lower-attenuation and appeared along the aorta wall. Displaced intimal calcifications were seen in nine of fifteen patients. There was no intimal flap on all five aortogram, while aortic wall thickening and atherosclerotic change were demonstrated in four cases and in one case, respectively. Focal ulcers were seen in three cases. Ulcer-like intimal defects were demonstrated in a total of eleven cases (eight on CT, two on aortogram, and one on both). In ten of the twelve cases seen on follow up CT, the thickness of the intramural hematoma was seen to be reduced. Among the 15 cases, the operation was performed in two cases, and the remaining 13 received conservative treatment. In ten cases observed for more than twelve months, a recurrence of symptoms did not occur. Eccentric aortic wall thickening in patients who complain of acute chest pain is the result of acute aortic dissection with intramural hematoma, or a penetrating atherosclerotic ulcer of the aorta. The later may be differentiated from the former by the presence of on ulcer-like intimal defect. When both diseases are limited to the descending aorta, conservative treatment may

  18. 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.

  19. Multiple idiopathic arterial aneurysms masquerading as aortic dissection

    OpenAIRE

    Naha, Kushal; Vivek, G; Shetty, Ranjan K; Dias, Lorraine Simone

    2013-01-01

    We report the case of a 58-year-old lady who presented with abdominal pain and backache. Although initial evaluation was strongly suggestive of abdominal aortic dissection, she was ultimately found to have multiple arterial aneurysms. Work-up for underlying vasculitis was negative. Surgical repair was planned and the patient was referred to a cardiovascular surgeon. This case highlights the importance of careful radiological assessment in patients with suspected aortic dissection.

  20. Acute abdominal aortic thrombosis caused by paroxysmal atrial fibrillation.

    Science.gov (United States)

    Riccioni, G; Bucciarelli, V; Bisceglia, N; Totaro, G; Scotti, L; Aceto, A; Martini, F; Gallina, S; Bucciarelli, T; Macarini, L

    2013-01-01

    Acute abdominal aortic thrombosis is a rare and potential fatal event, which occurs in adult subjects. We present the case of a 72-year-old-man, who referred to the emergency Department of our hospital because of persistent severe abdominal and perineal pain. Doppler ultrasounds and computerized tomography angiography revealed the acute thrombosis of the abdominal aorta. Immediate revascularization through aortic thrombo-endoarterectomy resolved the disease. PMID:23830410

  1. Coronary Ostial Stenosis after Aortic Valve Replacement

    Science.gov (United States)

    Ziakas, Antonios G.; Economou, Fotios I.; Charokopos, Nicholas A.; Pitsis, Antonios A.; Parharidou, Despina G.; Papadopoulos, Thomas I.; Parharidis, Georgios E.

    2010-01-01

    Coronary ostial stenosis is a rare but potentially serious sequela after aortic valve replacement. It occurs in the left main or right coronary artery after 1% to 5% of aortic valve replacement procedures. The clinical symptoms are usually severe and may appear from 1 to 6 months postoperatively. Although the typical treatment is coronary artery bypass grafting, patients have been successfully treated by means of percutaneous coronary intervention. Herein, we present the cases of 2 patients in whom coronary ostial stenosis developed after aortic valve replacement. In the 1st case, a 72-year-old man underwent aortic valve replacement and bypass grafting of the saphenous vein to the left anterior descending coronary artery. Six months later, he experienced a non-ST-segment-elevation myocardial infarction. Coronary angiography revealed a critical stenosis of the right coronary artery ostium. In the 2nd case, a 78-year-old woman underwent aortic valve replacement and grafting of the saphenous vein to an occluded right coronary artery. Four months later, she experienced unstable angina. Coronary angiography showed a critical left main coronary artery ostial stenosis and occlusion of the right coronary artery venous graft. In each patient, we performed percutaneous coronary intervention and deployed a drug-eluting stent. Both patients were asymptomatic on 6-to 12-month follow-up. We attribute the coronary ostial stenosis to the selective ostial administration of cardioplegic solution during surgery. We conclude that retrograde administration of cardioplegic solution through the coronary sinus may reduce the incidence of postoperative coronary ostial stenosis, and that stenting may be an efficient treatment option. PMID:20844624

  2. 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.

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

    DEFF Research Database (Denmark)

    Brunkwall, J; Kasprzak, P; Verhoeven, E; Heijmen, R; Taylor, P; Alric, P; Canaud, L; Janotta, M; Raithel, D; Malina, W; Resch, Ti; Eckstein, H-H; Ockert, S; Larzon, T; Carlsson, F; Schumacher, H; Classen, S; Schaub, P; Lammer, J; Lönn, Lars Birger; Clough, R E; Rampoldi, V; Trimarchi, S; Fabiani, J-N; Böckler, D; Kotelis, D; von Tenng-Kobligk, H; Mangialardi, N; Ronchey, S; Dialetto, G; Matoussevitch, V

    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...

  4. Ophthalmologic manifestations of focal dermal hypoplasia (Goltz syndrome): A case series of 18 patients.

    Science.gov (United States)

    Gisseman, Jordan D; Herce, Honey H

    2016-03-01

    Focal Dermal Hypoplasia (FDH) or Goltz syndrome is a rare multi-system disorder with cutaneous, ocular, dental, and skeletal anomalies due to dysplasia of mesoectodermal derived tissues. It is an X-linked inheritance syndrome caused by mutations in the PORCN gene. This study is aimed to investigate the ocular findings in patients with Goltz syndrome. To date, there have been a limited number of case reports on the ocular manifestations of FDH. This is a prospective, non-consecutive, observational case series. Prospective ophthalmologic evaluation was performed on 18 patients with confirmed genetic testing for FDH, Goltz Syndrome, as a component of a larger multi-subspecialty study to better characterize the findings of this condition. Special attention was placed on evaluating the incidence of anophthalmia, microphthalmia, colobomas (iris, optic nerve, and/or retinal), cataracts, nystagmus, and strabismus. A complete ophthalmologic exam was done on all the patients. The mean patient age was 12.8 years (1-55 years). Eighty-nine percent were female and 77% (14/18) of patients had some form of an ophthalmologic manifestation of the condition. Ophthalmological findings included chorioretinal colobomas (61%), iris colobomas (50%), microphthalmia (44%), anophthalmia (11%), cataracts (11%), and conjunctival and eyelid papillomas (5%). Nystagmus was present in 33% and strabismus in 22% of the patients. Visual acuity ranged from 20/20 to no light perception. This study demonstrates a higher incidence of ophthalmologic manifestations as previously reported (77% vs. 40%). To our knowledge, this is the largest case series reported in the literature with 18 patients. © 2016 Wiley Periodicals, Inc. PMID:27001926

  5. Tobacco smoking and aortic aneurysm

    DEFF Research Database (Denmark)

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

    2012-01-01

    from the Copenhagen City Heart Study followed for up to 34years and in 56,211 individuals from the Copenhagen General Population Study followed for up to 7years. RESULTS: During follow-up, 335 and 169 individuals developed aortic aneurysm outcomes in the Copenhagen City Heart Study and Copenhagen...... 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...

  6. A sinister cause of anterograde amnesia: painless aortic dissection.

    Science.gov (United States)

    April, Michael D; Fossum, Kurt; Hounshell, Charles; Stolper, Katherine; Spear, Leigh; Semelrath, Kevin

    2015-07-01

    Aortic dissection is a frequently devastating diagnosis classically associated with severe chest pain.We present a case of painless aortic dissection with anterograde amnesia. An 84-year-old man was brought to the emergency department by ambulance, when his wife noted that he developed acute onset complete loss of short-term memory. Medical history was notable for a 4.5-cm fusiform thoracic aortic root aneurysm. On arrival,he denied pain or syncope.On examination, he was mildly hypotensive(110/59 mm Hg); and there were no murmurs, pulse deficits, or focal neurologic deficits. During his stay, he developed left flank pain. Chest radiography demonstrated subtle mediastinal widening and obscuration of the aortic knob compared with previous films. Computed tomography revealed an extensive intimal flap consistent with an aortic dissection involving the sinus of Valsalva and left renal artery. The patient subsequently developed acute onset chest pain after which he became unresponsive. Echocardiography demonstrated tamponade physiology.The family decided to transition to comfort care measures, and the patient died soon after.We identified 7 other cases in the literature of aortic dissection cases with presentations consistent with transient global amnesia,5 of which without neurologic deficits and 3 of which without pain. This case highlights the imperative of a thorough history and high index of suspicion for this catastrophic diagnosis in patients with transient global amnesia who otherwise might be expected to have an excellent prognosis and little need for diagnostic work-up. PMID:25649752

  7. 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...

  8. 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...

  9. 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.)

  10. 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.)

  11. 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

  12. A Perspective Review on Numerical Simulations of Hemodynamics in Aortic Dissection

    Directory of Open Access Journals (Sweden)

    Wan Naimah Wan Ab Naim

    2014-01-01

    Full Text Available Aortic dissection, characterized by separation of the layers of the aortic wall, poses a significant challenge for clinicians. While type A aortic dissection patients are normally managed using surgical treatment, optimal treatment strategy for type B aortic dissection remains controversial and requires further evaluation. Although aortic diameter measured by CT angiography has been clinically used as a guideline to predict dilation in aortic dissection, hemodynamic parameters (e.g., pressure and wall shear stress, geometrical factors, and composition of the aorta wall are known to substantially affect disease progression. Due to the limitations of cardiac imaging modalities, numerical simulations have been widely used for the prediction of disease progression and therapeutic outcomes, by providing detailed insights into the hemodynamics. This paper presents a comprehensive review of the existing numerical models developed to investigate reasons behind tear initiation and progression, as well as the effectiveness of various treatment strategies, particularly the stent graft treatment.

  13. Unusual Case of Overt Aortic Dissection Mimicking Aortic Intramural Hematoma.

    Science.gov (United States)

    Disha, Kushtrim; Kuntze, Thomas; Girdauskas, Evaldas

    2016-04-01

    We report an interesting case in which overt aortic dissection mimicked two episodes of aortic intramural hematoma (IMH) (Stanford A, DeBakey I). This took place over the course of four days and had a major influence on the surgical treatment strategy. The first episode of IMH regressed completely within 15 hours after it was clinically diagnosed and verified using imaging techniques. The recurrence of IMH was detected three days thereafter, resulting in an urgent surgical intervention. Overt aortic dissection with evidence of an intimal tear was diagnosed intraoperatively. PMID:27066437

  14. 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.

  15. 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.

  16. 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

  17. [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

  18. Congenital porencephaly with cerebellar hypoplasia in a Holstein calf: a case report

    International Nuclear Information System (INIS)

    We describe the case of a nine-day-old female Holstein calf which had cheiloschisis, a moderate dome-shaped head, ataxia and opisthotonus since birth. No significant findings except the dome-shaped head were observed on survey radiography of the skull. Computed tomography (CT) images showed bilateral lateral ventriculomegaly, cerebellar hypoplasia and a cyst-like lesion communicating with the right lateral ventricle. Post-mortem examination revealed a cerebral defect in the frontoparietal lobe, which communicated with the right lateral ventricle, and cerebellar hypoplasia. CT provided a characteristic finding of porencephaly and was helpful for diagnosing the accompanying anomalies. We suggest that porencephaly should be included as a specific anomaly in the differential diagnosis of congenital brain malformation

  19. 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

  20. 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

  1. 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

  2. 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-...

  3. Tigroid pattern of the white matter: a previously unrecognized MR finding in lissencephaly with cerebellar hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kono, Tatsuo [Dokkyo University, Department of Radiology, Mibu, Shimotsuga, Tochigi (Japan); Moriyama, Nobuko [Ibaraki Children' s Hospital, Department of Paediatrics, Mito, Ibaraki (Japan); Tanaka, Ryuta [University of Tsukuba, Department of Paediatrics, Tsukubu, Ibaraki (Japan); Iwasaki, Nobuaki [Ibaraki Prefectural University of Health Sciences, Department of Paediatrics, Ami, Ibaraki (Japan); Arai, Jun-ichi [Ibaraki Children' s Hospital, Department of Neonatology, Mito, Ibaraki (Japan)

    2008-10-15

    Brain MR images of a 14-month-old boy with lissencephaly and cerebellar hypoplasia showed numerous radiating linear structures in the white matter. This finding was identical to the tigroid or leopard-skin pattern that is seen in Pelizaeus-Merzbacher disease or metachromatic leukodystrophy and represents the perivascular white matter spared from demyelination. We speculate that mutations of the reelin gene, expressed both in the cortex and in the white matter, may play an important role in its development. (orig.)

  4. 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

  5. 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

  6. 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 ...

  7. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... to requiring surgery for aortic stenosis, left ventricular hypertrophy is a common manifestation. By that, I mean generally speaking any muscle that works harder in the body gets thicker ...

  8. 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 ...

  9. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... thank you, Jim. This is obviously just a model of the heart just to give some perspective ... aortic repair, he would have had a cardiac evaluation prior to that and they probably would have ...

  10. 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 ...

  11. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... in severe aortic stenosis, as it's probably more stress than the patient's heart should have to undergo. ... There's been a lot of work done by academic cardiac surgeons and cardiologists to try to define ...

  12. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... other cardiovascular diseases including heart attack and increased risk of cardiovascular death. John, is there anything you ... with time. This highlights the mortality or the risk of death associated with aortic valve replacement and ...

  13. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... aortic stenosis and even tricuspid stenosis from rheumatic causes are much more common. To expand upon that ... out of the bloodstream and is thought to cause less untoward effects to the brain and other ...

  14. Minimally invasive aortic valve replacement

    DEFF Research Database (Denmark)

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

    2009-01-01

    . The 30-day mortality rate for the 98 patients was zero, although 14 of the 98 mini-sternotomies had to be converted to complete sternotomies intraoperatively due to technical problems. Such conversion doubled the operative time over that of the planned full sternotomies. In the group of patients whose......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 < 0.001), with the intended full-sternotomy group having the shortest times. In conclusion, the mini-aortic valve replacement is an...

  15. 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 ... for patients who would not tolerate well a traditional open operation or a less invasive operation, as ...

  16. 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 ... likely than patients who don't have other cardiovascular conditions: coronary artery disease, peripheral vascular disease, et ...

  17. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... in severe aortic stenosis, as it's probably more stress than the patient's heart should have to undergo. ... these series, when you critically look at the literature, the operative times can be significantly longer and ...

  18. 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 ...

  19. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... starts out as a disease we call aortic sclerosis, which is the beginning of the process of ... path life. Again, it's a progressive disease from sclerosis, or the beginning of thickening of the valve, ...

  20. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... thank you, Jim. This is obviously just a model of the heart just to give some perspective ... the aorta at this point. 9 Earlier this month Robin Williams had his aortic valve replaced and ...

  1. 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...

  2. 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 ...

  3. 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 ... surgery we, as surgeons, know from our anatomy training during our course of training, we know where ...

  4. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... John, is there anything you can do to help prevent aortic stenosis? There's no prophylactic methods that ... very good looks. It's a great tool to help cardiac surgeons. Thanks, Jim. And I would underscore ...

  5. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... able to look at the aortic valve very well. In fact, transesophageal echocardiography is the best modality ... use to help evaluate both cardiac function as well as anatomy in patients who need heart surgery. ...

  6. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... a series of charts that we have, historical data, to know really what the best size is ... heart and lung circulation, so that we can open the aorta safely and replace this patient's aortic ...

  7. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... in severe aortic stenosis, as it's probably more stress than the patient's heart should have to undergo. ... in younger patients who want to return to work and activity more quickly, we feel that this ...

  8. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... uncommon to have more problems with their tissue quality and so we do have to be careful ... does not have an aortic aneurysm in the first part of their aorta there, their ascending aorta. ...

  9. 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, ...

  10. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... This process starts out as a disease we call aortic sclerosis, which is the beginning of the ... either tissues from cows or pigs, what we call porcine for pigs and bovine for cows. Essentially, ...

  11. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... including heart attack and increased risk of cardiovascular death. John, is there anything you can do to ... aortic stenosis, there's a pretty rapid progression into death if no intervention is taken. We feel that ...

  12. 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

  13. 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.

  14. 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

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

    International Nuclear Information System (INIS)

    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/m2 and 24.2 ± 5.3 mm/m2, respectively) and raphe+ (17.3 ± 2.2 mm/m2 and 24.2 ± 5.5 mm/m2, respectively) were significantly different from those with BAV-LA (15.8 ± 1.9 mm/m2 and 26.4 ± 5.5 mm/m2, respectively) and raphe- (15.7 ± 1.9 mm/m2 and 26.2 ± 5.4 mm/m2, 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.)

  16. 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.)

  17. 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 ...

  18. 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

  19. Intramural hematoma or aortic dissection – a diagnostic and therapeutic problem. A case report

    Science.gov (United States)

    Suder, Bogdan; Wasilewski, Grzegorz; Sadowski, Jerzy; Kapelak, Bogusław

    2015-01-01

    The authors present a case report of a 60-year-old patient with an ascending aortic aneurysm along with the associated diagnostic and therapeutic problems. The choice of therapy in patients with aortic intramural hematoma is difficult and should be based on comprehensive evaluation of the patient's status as well as on the experience of the radiologist and surgeon. PMID:26702280

  20. Ultrasonographic diagnosis of aortic thrombosis after umbilical artery catheterization in neonate

    International Nuclear Information System (INIS)

    We present two cases of aortic thrombosis which developed after umbilical artery catheterization. Aortic thrombosis, one of the complications of umbilical artery catheterization can easily be diagnosed by ultrasonography, so ultrasonography would be needed as a screening study in neonate with umbilical artery catheterization.

  1. 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

  2. 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

  3. Salvage Extracorporeal Membrane Oxygenation Prior to "Bridge" Transcatheter Aortic Valve Replacement.

    Science.gov (United States)

    Chiu, Peter; Fearon, William F; Raleigh, Lindsay A; Burdon, Grayson; Rao, Vidya; Boyd, Jack H; Yeung, Alan C; Miller, David Craig; Fischbein, Michael P

    2016-06-01

    We describe a patient who presented in profound cardiogenic shock due to bioprosthetic aortic valve stenosis requiring salvage Extracorporeal Membrane Oxygenation followed by a "bridge" valve-in-valve transcatheter aortic valve replacement. doi: 10.1111/jocs.12750 (J Card Surg 2016;31:403-405). PMID:27109017

  4. 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.

  5. Chest CT scanning for clinical suspected thoracic aortic dissection: beware the alternate diagnosis.

    Science.gov (United States)

    Thoongsuwan, Nisa; Stern, Eric J

    2002-11-01

    The aim of the study was retrospectively to evaluate the spectrum of chest diseases in patients presenting with clinical suspicion of thoracic aortic dissection in the emergency department. We performed a retrospective medical records review of 86 men and 44 women (ages ranging between 23 and 106 years) with clinically suspected aortic dissection, for CT scan findings and final clinical diagnoses dating between January 1996 and September 2001. All images were obtained by using a standard protocol for aortic dissection. We found aortic dissection in 32 patients (24.6%), 22 of which were Stanford classification type A and 10 Stanford type B. In 70 patients (53.9%), chest pain could not be explained by the CT scan findings. However, in 28 patients (21.5%), CT scanning did reveal an alternate diagnosis that, along with the clinical impression, probably explained the patients' presenting symptoms, including: hiatal hernia (7), pneumonia (5), intrathoracic mass (4), pericardial effusion/hemopericardium (3), esophageal mass/rupture (2), aortic aneurysm without dissection (2), pulmonary embolism (2), pleural effusion (1), aortic rupture (1), and pancreatitis (1). In cases where there is clinical suspicion of aortic dissection, CT scan findings of an alternate diagnosis for the presenting symptoms are only slightly less common than the finding of aortic dissection itself. Although the spectrum of findings will vary depending upon your patient population, beware the alternate diagnosis. PMID:15290550

  6. 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

  7. Endoluminal treatment of aortic dissection

    Energy Technology Data Exchange (ETDEWEB)

    Chavan, Ajay; Lotz, Joachim; Galanski, Michael [Department of Diagnostic Radiology, Hannover Medical School, Carl Neuberg Strasse 1, 30625, Hannover (Germany); Oelert, Frank; Haverich, Axel; Karck, Matthias [Department of Thoracic and Cardiovascular Surgery, Hannover Medical School, Carl Neuberg Strasse 1, 30625, Hannover (Germany)

    2003-11-01

    Aortic dissection is most often a catastrophic medical emergency which, if untreated, can be potentially fatal. The intention of therapy in patients with aortic dissection is to prevent aortic rupture or aneurysm formation as well as to relieve branch vessel ischaemia. Patients with aortic dissection are often poor candidates for anaesthesia and surgery and the surgical procedure itself is challenging requiring thoracotomy, aortic cross clamping, blood transfusion as well as prolonged hospital stay in some cases. Operative mortality is especially high in patients with critical mesenteric or renal ischaemia. The past decade has experienced the emergence of a number of interventional radiological or minimally invasive techniques which have significantly improved the management of patients with aortic dissection. These include stent grafting for entry site closure to prevent aneurysmatic widening of the false lumen as well as percutaneous techniques such as balloon fenestration of the intimal flap and aortic true lumen stenting to alleviate branch vessel ischaemia. False lumen thrombosis following entry closure with stent grafts has been observed in 86-100% of patients, whereas percutaneous interventions are able to effectively relieve organ ischaemia in approximately 90% of the cases. In the years to come, it is to be expected that these endoluminal techniques will become the method of choice for treating most type-B dissections and will assist in significantly reducing the number of open surgical procedures required for type-A dissections. The intention of this article is to provide an overview of the current status of these endoluminal techniques based on our own experience as well as on a review of the relevant literature. (orig.)

  8. Endoluminal treatment of aortic dissection

    International Nuclear Information System (INIS)

    Aortic dissection is most often a catastrophic medical emergency which, if untreated, can be potentially fatal. The intention of therapy in patients with aortic dissection is to prevent aortic rupture or aneurysm formation as well as to relieve branch vessel ischaemia. Patients with aortic dissection are often poor candidates for anaesthesia and surgery and the surgical procedure itself is challenging requiring thoracotomy, aortic cross clamping, blood transfusion as well as prolonged hospital stay in some cases. Operative mortality is especially high in patients with critical mesenteric or renal ischaemia. The past decade has experienced the emergence of a number of interventional radiological or minimally invasive techniques which have significantly improved the management of patients with aortic dissection. These include stent grafting for entry site closure to prevent aneurysmatic widening of the false lumen as well as percutaneous techniques such as balloon fenestration of the intimal flap and aortic true lumen stenting to alleviate branch vessel ischaemia. False lumen thrombosis following entry closure with stent grafts has been observed in 86-100% of patients, whereas percutaneous interventions are able to effectively relieve organ ischaemia in approximately 90% of the cases. In the years to come, it is to be expected that these endoluminal techniques will become the method of choice for treating most type-B dissections and will assist in significantly reducing the number of open surgical procedures required for type-A dissections. The intention of this article is to provide an overview of the current status of these endoluminal techniques based on our own experience as well as on a review of the relevant literature. (orig.)

  9. 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.

  10. 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.

  11. Pulmonary hypoplasia on preterm infant associated with diffuse chorioamniotic hemosiderosis caused by intrauterine hemorrhage due to massive subchorial hematoma: report of a neonatal autopsy case.

    Science.gov (United States)

    Yamada, Sohsuke; Marutani, Takamitsu; Hisaoka, Masanori; Tasaki, Takashi; Nabeshima, Atsunori; Shiraishi, Mika; Sasaguri, Yasuyuki

    2012-08-01

    A male infant born prematurely at 31 weeks of gestation weighed 789 g and had mildly brown-colored oral/tracheal aspirates at delivery. The amniotic fluid was also discolored, and its index was below 5. The patient died of hypoxemic respiratory and cardiac failure 2 hours after birth. The maternal profiles showed placenta previa and intrauterine growth restriction (IUGR) at 22 weeks of gestation, and revealed recurrent episodes of antenatal and substantial vaginal bleeding and oligohydramnios, indicating chronic abruption-oligohydramnios sequence. The thickened placenta, weighing 275 g, grossly displayed unevenness and diffuse opacity with green to brown discoloration in the chorioamniotic surface, and revealed chronic massive subchorial hematomas (Breus' mole) with old peripheral blood clot, circumvallation, and infarction. Microscopically, diffuse Berlin-blue staining-positive hemosiderin deposits were readily encountered in the chorioamniotic layers of the chorionic plate, consistent with diffuse chorioamniotic hemosiderosis (DCH) due to Breus' mole, accompanied by diffuse amniotic necrosis. At autopsy, an external examination showed several surface anomalies and marked pulmonary hypoplasia, 0.006 (less 0.012) of lung:body weight ratio. Since Breus' mole has a close relationship with intrauterine hemorrhage, resulting in DCH, IUGR, and/or pulmonary hypoplasia of the newborn, the present features might be typical. PMID:22827763

  12. A Novel Technique To Correct Multiplanar Maxillary Hypoplasia.

    Science.gov (United States)

    Simon, Sibu Sajjan; Charlu, Arun Paul; Chacko, Rabin Kurudamannil; Kumar, Saurabh

    2016-04-01

    Dental malocclusion and facial deformity are frequent observations in patients with clefts of the orofacial region. These patients have a low self perception secondary to their aesthetic appearance. Cleft palate patients are further affected in their speech and oral function with direct impediment to their quality of life. Early identification and treatment in cleft lip and palate patients may directly enhance their overall well being and productivity with sustainable prognosis when managed by skilled and evidence informed operators. We present a successful case management of a patient with a cleft palate and dentofacial deformity with a past surgical history, treated with an anterior maxillary advancement osteotomy, stabilized with an interpositional non vascular iliac bone graft. The posterior open bite was corrected using overlay full coverage crowns. Both these techniques are rarely reported in the literature. The procedure positively improved the quality of life in our patient with regards to her aesthetics, speech and function. This treatment approach could be considered in similar cases to achieve predictable outcomes. PMID:27190966

  13. A Novel Technique To Correct Multiplanar Maxillary Hypoplasia

    Science.gov (United States)

    Charlu, Arun Paul; Chacko, Rabin Kurudamannil; Kumar, Saurabh

    2016-01-01

    Dental malocclusion and facial deformity are frequent observations in patients with clefts of the orofacial region. These patients have a low self perception secondary to their aesthetic appearance. Cleft palate patients are further affected in their speech and oral function with direct impediment to their quality of life. Early identification and treatment in cleft lip and palate patients may directly enhance their overall well being and productivity with sustainable prognosis when managed by skilled and evidence informed operators. We present a successful case management of a patient with a cleft palate and dentofacial deformity with a past surgical history, treated with an anterior maxillary advancement osteotomy, stabilized with an interpositional non vascular iliac bone graft. The posterior open bite was corrected using overlay full coverage crowns. Both these techniques are rarely reported in the literature. The procedure positively improved the quality of life in our patient with regards to her aesthetics, speech and function. This treatment approach could be considered in similar cases to achieve predictable outcomes. PMID:27190966

  14. 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.)

  15. Aortic valve replacement through right thoracotomy.

    OpenAIRE

    Rao, P N; A S Kumar

    1993-01-01

    There has never, to our knowledge, been a report of aortic valve replacement via a right thoracotomy. However, we recently used this approach in 2 young women with severe aortic stenosis. Exposure of the aortic valve was excellent, and we encountered neither technical difficulties nor sequelae related to the right thoracotomy. We believe that right thoracotomy provides adequate access for safe aortic valve replacement and yields cosmetically more appealing results than does median sternotomy.

  16. 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.

  17. Resolution of an Acute Aortic Syndrome with Aortic Valve Insufficiency Post-PCI

    OpenAIRE

    de Barros e Silva, Pedro G.M.; Aquino, Thiago de; Resende, Marcos V.; Richter, Ivo; Barros, Cecilia M.; Andrioli, Vanessa G.; Baruzzi, Antonio C.; Medeiros, Caio C.J.; Furlan, Valter

    2014-01-01

    Patient: Female, 52 Final Diagnosis: Acute aortic syndrome with aortic valve insufficiency post-PCI Symptoms: Chest pain Medication: — Clinical Procedure: Conservative Specialty: Cardiology Objective: Unusual or unexpected effect of treatment Background: Acute aortic syndrome is the modern term that includes aortic dissection, intramural hematoma, and symptomatic aortic ulcer. Iatrogenic coronary artery dissection extending to the aorta during percutaneous coronary intervention is a very rare...

  18. 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.

  19. IMAGe association: report of two cases in siblings with adrenal hypoplasia and review of the literature.

    Science.gov (United States)

    Phillips, Katherine; Arroyo, May R; Duckworth, Lizette Vila

    2014-01-01

    We report the postmortem findings of two siblings with gross and microscopic features consistent with IMAGe association (Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital anomalies) with an emphasis on the histopathology of the adrenal gland in this rare syndrome. The first sibling was an 8-week old male diagnosed postnatally with primary adrenal insufficiency. There was no deletion of the DAX1 gene by FISH. Examination at autopsy revealed dysmorphic features including frontal bossing, epicanthal folds, flat philtrum, cryptorchidism, penile chordee, overriding fourth toe, and height and weight below 3rd percentile. Grossly, the adrenal glands were not identified; however, microscopic examination of the suprarenal soft tissue revealed a 3 mm focus of disorganized fetal adrenal cortex with distended "cytomegalic" cells with abundant pink eosinophilic cytoplasm, vesicular nuclei, and cytoplasmic vacuolization. A minute focus of permanent adult cortex was also seen, but no adrenal medulla was identified. An autopsy of the sibling, who died 12 years previously at day 9 of life, revealed dysmorphic facial features with cryptorchidism and a large phallus. The adrenal glands were grossly hypoplastic (11 mm). Histologically, the adrenal glands showed disorganized fetal cortex with cytomegalic cells, a larger amount of permanent adult cortex, and bizarre nuclei with numerous pseudoinclusions. While there is currently limited information regarding the histopathologic adrenal findings in IMAGe association, our small case series suggests overlapping features between X-linked recessive congenital adrenal hypoplasia (cytomegalic cells with lack of permanent adult cortex) and autosomal recessive congenital adrenal hypoplasia (diminished permanent adult cortex without cytomegalic cells). PMID:24617583

  20. Stroke in Patients With Aortic Stenosis

    DEFF Research Database (Denmark)

    Greve, Anders Møller; Dalsgaard, Morten; Bang, Casper N;

    2014-01-01

    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 graftin...... associated with mortality. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT00092677....

  1. Persistent Fifth Aortic Arch with Coarctation

    Science.gov (United States)

    Kim, Sue Hyun; Choi, Eun-Suk; Cho, Sungkyu; Kim, Woong-Han

    2016-01-01

    Persistent fifth aortic arch (PFAA) is a rare congenital anomaly of the aortic arch frequently associated with other cardiovascular anomalies, such as tetralogy of Fallot and aortic arch coarctation or interruption. We report the case of a neonate with PFAA with coarctation who successfully underwent surgical repair. PMID:26889445

  2. [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

  3. 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).

  4. [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

  5. 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

  6. 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, ...

  7. 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.

  8. 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...

  9. Myocardial Infarction in a Patient with Prosthetic Aortic Valve

    Directory of Open Access Journals (Sweden)

    Seyed Kianoosh Hoseini

    2006-08-01

    Full Text Available A 45- year old man with a history of Aortic Valve replacement presented with acute chest pain which was diagnosed to be anterior wall myocardial infarction. He received thrombolytic therapy with streptokinase. Echocardiography and fluoroscopy showed normally functioning ball and cage aortic prosthesis. Coronary arteriography showed globular filling defect in midportion of left anterior descending coronary artery, most probably embolized thrombus. The patient underwent medical treatment especially warfarin with higher range of INR without any intervention. He had a smooth in-hospital course and uneventful recovery.

  10. Intracardiac echocardiography to diagnose pannus formation after aortic valve replacement.

    Science.gov (United States)

    Yamamoto, Yoshiya; Ohara, Takahiro; Funada, Akira; Takahama, Hiroyuki; Amaki, Makoto; Hasegawa, Takuya; Sugano, Yasuo; Kanzaki, Hideaki; Anzai, Toshihisa

    2016-03-01

    A 66-year-old female, under regular follow-up for 20 years after aortic valve replacement (19-mm Carbomedics), presented dyspnea on effort and hypotension during hemodialysis. A transthoracic echocardiogram showed elevation of transvalvular velocity up to 4 m/s, but the structure around the aortic prosthesis was difficult to observe due to artifacts. Fluoroscopy revealed normal motion of the leaflets of the mechanical valve. Intracardiac echocardiography (ICE) revealed a pannus-like structure in the left ventricular outflow tract. Transesophageal echocardiogram also revealed this structure. ICE can visualize structural abnormalities around a prosthetic valve after cardiac surgery even in patients in whom conventional imaging modalities failed. PMID:26732266

  11. Aortic Impedance in Little Mice

    OpenAIRE

    Reddy, Anilkumar K.; Taffet, George E.; Hartley, Craig J.

    2008-01-01

    The Little dwarf mouse lives 30% longer than its age-matched wild-type (WT) mouse. We determined aortic input impedance in 21 (8 Little, 13 WT) 4 month-old mice. Modulus of impedance was calculated from the Fourier transformed aortic pressure (P) and average luminal flow velocity (Vavg) as ∣Zi∣ = ∣P∣/∣Vavg∣. Characteristic impedance was estimated by averaging the 2nd-10th harmonic of the impedance moduli. We found the impedance modulus ∣Zi∣ to be similar in the 2 groups (WT vs. Little; mean±S...

  12. Discrepancy in compliance between the clinical and genetic diagnosis of choroidal hypoplasia in Danish Rough Collies and Shetland Sheepdogs.

    Science.gov (United States)

    Fredholm, M; Larsen, R C; Jönsson, M; Söderlund, M A; Hardon, T; Proschowsky, H F

    2016-04-01

    Collie eye anomaly (CEA) is a congenital, inherited ocular disorder which is widespread in herding breeds. Clinically, the two major lesions associated with CEA are choroidal hypoplasia (CH) and coloboma, and both lesions are diagnosed based on ophthalmological examination. A 7.8-kb intronic deletion in the gene encoding non-homologous end-joining factor 1 (NHEJ1) has been reported to be the causative mutation underlying CH when present in the homozygous state. In this study, we have investigated the compliance between the clinical and genetic diagnosis of CH in the Danish Rough Collie and Shetland Sheepdog populations. Our results show that the deletion in NHEJ1 is not predictive for CH in the Danish Rough Collie population, whereas the clinical and genetic diagnosis is in accordance with each other in the Shetland Sheepdog population. Based on these results, it can be concluded that the intronic deletion in NHEJ1 is not the causative mutation but, rather, a marker linked to the locus underlying the trait in some populations but linked to both the wild-type and CH-causing locus in most dogs in the Danish Rough Collie population. PMID:26732749

  13. Treatment of an Aortic Traumatic Double Rupture

    Directory of Open Access Journals (Sweden)

    Attinà Domenico

    2015-03-01

    Full Text Available Traumatic thoracic aortic rupture is a life-threatening condition; aortic isthmus is the most common site of rupture, but in rare cases traumatic injury can localize elsewhere, such as at aortic arch or at the level of the diaphragm. In the past few years, endovascular treatment of traumatic aortic injury became a safe procedure, with lower mortality and complication, if compared with open surgery. We report a case of a 40-year-old-man admitted to emergency department after a violent car crash in which an aortic traumatic double rupture was successfully treated with two endovascular stent-grafts coverage.

  14. 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

  15. 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...

  16. 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

  17. 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

  18. [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

  19. 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

  20. 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

  1. 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.

  2. 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.

  3. 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.

  4. Abdominal aortic surgery and renal anomalies

    Directory of Open Access Journals (Sweden)

    Ilić Nikola

    2011-01-01

    Full Text Available Introduction. Kidney anomalies present a challenge even for the most experienced vascular surgeon in the reconstruction of the aortoilliac segment. The most significant anomalies described in the surgery of the aortoilliac segment are a horse-shoe and ectopic kidney. Objective. The aim of this retrospective study was to analyze experience on 40 patients with renal anomalies, who underwent surgery of the aortoilliac segment and to determine attitudes on conventional surgical treatment. Methods. In the period from 1992 to 2009, at the Clinic for Vascular Surgery of the Clinical Centre of Belgrade we operated on 40 patients with renal anomalies and aortic disease (aneurysmatic and obstructive. The retrospective analysis involved standard epidemiological data of each patient (gender, age, risk factors for atherosclerosis, type of anomaly, type of aortic disease, presurgical parameter values of renal function, type of surgical approach (laparatomy or retroperitoneal approach, classification of the renal isthmus, reimplantation of renal arteries and perioperative morbidity and mortality. Results. Twenty patients were males In 30 (70% patients we diagnosed a horse-shoe kidney and in 10 (30% ectopic kidney. In the cases of ruptured aneurysm of the abdominal aorta the diagnosis was made by ultrasound findings. Pre-surgically, renal anomalies were confirmed in all patients, except in those with a ruptured aneurysm who underwent urgent surgery. In all patients we applied medial laparatomy, except in those with a thoracoabdominal aneurysm type IV, when the retroperitonal approach was necessary. On average the patients were under follow-up for 6.2 years (from 6 months to 17 years. Conclusion. Under our conditions, the so-called double clamp technique with the preservation of the kidney gave best results in the patients with renal anomalies and aortic disease.

  5. 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. ...

  6. 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 ...

  7. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... or in most patients who come to requiring surgery for aortic stenosis, left ventricular hypertrophy is a common manifestation. By that, I mean generally speaking any muscle that works harder in the body gets thicker and bigger over time and that's ...

  8. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... county more likely we see aortic stenosis. Again, patient education is part of the evaluation and management of valvular disease prior to surgery is paramount. It's important for a patient to understand really with any chronic condition what ...

  9. Minimally Invasive Aortic Valve Replacement

    Medline Plus

    Full Text Available ... valve in the aortic position will grow over time and proves a nice replacement. However, that's sort of operating on two valves to fix one valvular problem. And for most patients in our practice in their 50s, 60s, et cetera, we tend ...

  10. 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

  11. 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...

  12. Transapical transcatheter aortic valve implantation in a complex aortic surgical patient: A case involving the youngest valve-in-valve implantation with a 29 mm transcather valve

    OpenAIRE

    Attia, Rizwan; Visagan, Ravi; Nowell, Justin; Chadalavada, Sucharitha; Thomas, Martyn; Bapat, Vinne

    2012-01-01

    The present article reports a case involving a 29-year-old man who developed severe cardiac failure (New York Heart Association class IV). He had a complex surgical history, beginning with the repair of an anterior sinus of Valsalva aneurysm and closure of a ventricular septal defect at eight months of age. His residual Valsalva aneurysm and mixed aortic valve disease necessitated mechanical aortic valve replacement at 14 years of age. One year later, he developed coagulase-negative staphyloc...

  13. 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

  14. Chronic Otitis Media Resulting in Aortic Valve Replacement: A Case Report

    Directory of Open Access Journals (Sweden)

    Adem Guler

    2015-10-01

    Full Text Available The bicuspid aortic valve is known to be the most common congenital cardiac malformation, with an approximate incidence rate of 1-2% in the general population. Most patients are unaware of the disease until the onset of infective endocarditis, which is a life-threatening complication that may affect a heart valve or other cardiac structures at the site of endothelial damage. A 22-year-old man presented to our internal medicine clinic with a complaint of acute onset dyspnea and fatigue. His body temperature was 38 °C. A diastolic murmur was detected at the right sternal border. Two-dimensional transthoracic echocardiography revealed severe aortic insufficiency, and two-dimensional transesophageal echocardiography showed that the aortic valve was bicuspid. There was also a flail lesion extending the left ventricular outflow tract, resulting in pathological coaptation and severe aortic insufficiency. The patient was referred to our cardiovascular department for surgery. We herein present this case of a bicuspid aortic valve complicated by infective endocarditis due to the underlying disease of chronic otitis media related to a rare pathogen: Alloiococcus otitidis. The patient underwent a successful aortic valve replacement surgery due to aortic insufficiency following infective endocarditis. He was discharged on the 16th postoperative day in good condition.

  15. Chronic Otitis Media Resulting in Aortic Valve Replacement: A Case Report.

    Science.gov (United States)

    Guler, Adem; Sahin, Mehmet Ali; Gurkan Yesil, Fahri; Yildizoglu, Uzeyir; Demirkol, Sait; Arslan, Mehmet

    2015-04-01

    The bicuspid aortic valve is known to be the most common congenital cardiac malformation, with an approximate incidence rate of 1-2% in the general population. Most patients are unaware of the disease until the onset of infective endocarditis, which is a life-threatening complication that may affect a heart valve or other cardiac structures at the site of endothelial damage. A 22-year-old man presented to our internal medicine clinic with a complaint of acute onset dyspnea and fatigue. His body temperature was 38 (°)C. A diastolic murmur was detected at the right sternal border. Two-dimensional transthoracic echocardiography revealed severe aortic insufficiency, and two-dimensional transesophageal echocardiography showed that the aortic valve was bicuspid. There was also a flail lesion extending the left ventricular outflow tract, resulting in pathological coaptation and severe aortic insufficiency. The patient was referred to our cardiovascular department for surgery. We herein present this case of a bicuspid aortic valve complicated by infective endocarditis due to the underlying disease of chronic otitis media related to a rare pathogen: Alloiococcus otitidis. The patient underwent a successful aortic valve replacement surgery due to aortic insufficiency following infective endocarditis. He was discharged on the 16(th) postoperative day in good condition. PMID:26110009

  16. [Intermittent diastolic dysfunction of a mechanical aortic prosthesis: a clinical case].

    Science.gov (United States)

    Pinneri, F; De Felice, F; Gostoli, E; Garzaro, L; Mazza, A; Borello, G

    2000-09-01

    We describe the case of a 48 year-old man whose Sorin bileaflet aortic prosthesis presented persistent systolic and intermittent diastolic failure due to fibrous tissue overgrowth in the left ventricular outflow tract. PMID:11140292

  17. Prenatal diagnosis of tetralogy of Fallot with a double aortic arch.

    Science.gov (United States)

    Cheng, Andrew L; Pruetz, Jay D; Kung, Grace C

    2016-06-01

    In this study, we present a case of prenatally diagnosed tetralogy of Fallot with a double aortic arch, correlating images from fetal echocardiography, transthoracic echocardiography, and cardiac MRI. PMID:26983561

  18. 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.

  19. Discrepancy in compliance between the clinical and genetic diagnosis of choroidal hypoplasia in Danish Rough Collies and Shetland Sheepdogs

    DEFF Research Database (Denmark)

    Fredholm, Merete; Larsen, R. C.; Jönsson, M.; Söderlund, M. A.; Hardon, T.; Proschowsky, H. F.

    2016-01-01

    Collie eye anomaly (CEA) is a congenital, inherited ocular disorder which is widespread in herding breeds. Clinically, the two major lesions associated with CEA are choroidal hypoplasia (CH) and coloboma, and both lesions are diagnosed based on ophthalmological examination. A 7.8-kb intronic...

  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...

  1. 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

  2. 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.

  3. 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.

  4. 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. PMID:24057860

  5. 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...

  6. Early outcomes of transcatheter aortic valve replacement in patients with severe aortic stenosis: single center experience

    OpenAIRE

    Bozkurt, Engin; KELEŞ, TELAT; Durmaz, Tahir; Akçay, Murat; Sari, Cenk; Ayhan, Hüseyin; Bayram, Nihal Akar; Aslan, Abdullah Nabi; Baştuğ, Serdal; Bilen, Emine

    2014-01-01

    Introduction Transcatheter aortic valve implantation is a promising alternative to high risk surgical aortic valve replacement. The procedure is mainly indicated in patients with severe symptomatic aortic stenosis who cannot undergo surgery or who are at very high surgical risk. Aim Description early results of our single-center experience with balloon expandable aortic valve implantation. Material and methods Between July 2011 and August 2012, we screened in total 75 consecutive patients wit...

  7. 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.

  8. 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

  9. 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.

  10. Hypoplasia of the nose and eyes, hyposmia, hypogeusia, and hypogonadotrophic hypogonadism in two males.

    Science.gov (United States)

    Bosma, J F; Henkin, R I; Christiansen, R L; Herdt, J R

    1981-01-01

    Two males, 9-11 and 29-31 years of age, with severe hypoplasia of the nose, hypoplasia of the eyes, sensory abnormalities of taste and smell, and hypogonadism were studied. The nasal septum, cribriform plates and foramina of the vomeronasal (vn) nerves were demonstrated in both; the capsule of the vn organ was shown in one. Their nasal skeleton, demonstrated by tomoradiography, had grown in early embryological form. The nose was not patent in either patient. In both, the cranial vaults, orbits, epipharynges, and oral cavities were indented toward the hypoplastic nasal composite and the peripheral dimensions of their faces were normal for their respective ages. Each patient had impaired visual function with cataracts and colobomata. Each was unable to recognize the smell of any vapor (Type I hyposmia), and had severe impairment of recognition of any tastant (recognition hypogeusia); detection of vapors and of tastants were in appropriate anatomical areas. Each was unable orally to recognize standard plastic forms (astereognosis) though each could recognize the forms manually. Each patient had bilateral inguinal hernias, one or two undescended testes, and hypogonadotrophic hypogonadism. These patients do not fall within the spectrum of arrhinencephaly because of the presence of medial structure of attachment of the falx cerebri and because of their normal intelligence. Distinction of patients with this pattern of abnormalities from arrhinencephaly is important by reason of their potentiality of normal mental development. We hypothesize that their abnormalities resulted from an embryological disruption that occurred in the first trimester of pregnancy. The embryogenesis of the nasal composite is presumed to have been adequate for reciprocal induction of the anlagen of the forebrain. Development of their faces to normal peripheral dimensions indicates that the nasal composite is not essential for gross facial enlargement. PMID:6802865

  11. Phenotypic and Functional Changes of Endothelial and Smooth Muscle Cells in Thoracic Aortic Aneurysms

    Directory of Open Access Journals (Sweden)

    Anna Malashicheva

    2016-01-01

    Full Text Available Thoracic aortic aneurysm develops as a result of complex series of events that alter the cellular structure and the composition of the extracellular matrix of the aortic wall. The purpose of the present work was to study the cellular functions of endothelial and smooth muscle cells from the patients with aneurysms of the thoracic aorta. We studied endothelial and smooth muscle cells from aneurysms in patients with bicuspid aortic valve and with tricuspid aortic valve. The expression of key markers of endothelial (CD31, vWF, and VE-cadherin and smooth muscle (SMA, SM22α, calponin, and vimentin cells as well extracellular matrix and MMP activity was studied as well as and apoptosis and cell proliferation. Expression of functional markers of endothelial and smooth muscle cells was reduced in patient cells. Cellular proliferation, migration, and synthesis of extracellular matrix proteins are attenuated in the cells of the patients. We show for the first time that aortic endothelial cell phenotype is changed in the thoracic aortic aneurysms compared to normal aortic wall. In conclusion both endothelial and smooth muscle cells from aneurysms of the ascending aorta have downregulated specific cellular markers and altered functional properties, such as growth rate, apoptosis induction, and extracellular matrix synthesis.

  12. 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

  13. 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...

  14. Treatment options for postdissection aortic aneurysms.

    Science.gov (United States)

    Sobocinski, Jonathan; Patterson, Benjamin O; Clough, Rachel E; Spear, Rafaelle; Martin-Gonzalez, Teresa; Azzaoui, Richard; Hertault, Adrien; Haulon, Stéphan

    2016-04-01

    Aortic dissection is one of the most devastating catastrophes that can affect the aorta. Surgical treatment is proposed only when complications such as rupture or malperfusion occur. No clear consensus has been reached regarding the best therapy to prevent aortic rupture after the acute phase. We have performed a thorough review of the most recent literature on the strategies to treat patients in the chronic phase of aortic dissection. PMID:26771869

  15. 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.

  16. 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.

  17. 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 ...

  18. 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...

  19. Transverse Aortic Constriction in Mice

    OpenAIRE

    deAlmeida, Angela C.; van Oort, Ralph J.; Wehrens, Xander H. T.

    2010-01-01

    Transverse aortic constriction (TAC) in the mouse is a commonly used experimental model for pressure overload-induced cardiac hypertrophy and heart failure.1 TAC initially leads to compensated hypertrophy of the heart, which often is associated with a temporary enhancement of cardiac contractility. Over time, however, the response to the chronic hemodynamic overload becomes maladaptive, resulting in cardiac dilatation and heart failure.2 The murine TAC model was first validated by Rockman et ...

  20. 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

  1. 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.

  2. 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

  3. [Surgical aspects of acute aortic dissection].

    Science.gov (United States)

    Laas, J; Heinemann, M; Jurmann, M; Borst, H G

    1992-12-01

    This paper highlights some of the surgical aspects of acute aortic dissections such as: emergency diagnosis, indications for surgery, reconstructive operative techniques, malperfusion phenomena and necessity for follow-up. Aortic dissection is caused by an intimal tear, called the "entry", and subsequent splitting of the media by the stream of blood. Two lumina are thus created, which may communicate through "re-entries". As this creates severe weakness of the aortic wall, rupture and/or dilatation are the imminent dangers of acute aortic dissection. Acute aortic dissection type A, by definition involving the ascending aorta (Figures 1 and 2), is an absolute indication for emergency surgical treatment, because its natural history shows an extremely poor outcome (Figure 3). Due to impending (intrapericardial) aortic rupture, it may be necessary to limit diagnostic procedures to a minimum. Transesophageal echocardiography is the method of choice for establishing a quick, precise and reliable diagnosis (Figure 4). In stable patients, computed tomography gives additional information about aortic diameters or sites of extrapericardial perforation. Digital subtraction angiography (DSA) shows perfusion of the lumina and dependent organs. The surgical strategy in acute aortic dissection type A aims at replacement of the ascending aorta. Reconstructive techniques have to be considered, especially in aortic valve regurgitation without annuloectasia (Figures 5 and 6). In recent times, the use of GRF tissue glue has reduced the need for teflon felt. Involvement of the aortic arch should be treated aggressively up to the point of total arch replacement in deep hypothermic circulatory arrest as part of the primary procedure (Figure 7). Malperfusion phenomena of aortic branches remain risk-factors.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1483624

  4. 11 cm Haughton D left cervical aortic arch aneurysm

    OpenAIRE

    Kaul, Pankaj

    2013-01-01

    A 56 year old Caucasian man presented with sudden loss of consciousness while driving and was found to have an 11 cm Haughton D type left cervical aortic arch aneurysm with normal brachiocephalic branching and normal descending thoracic laterality but with considerable tortuosity and redundancy of aortic arch. The aneurysm arose between the left common carotid artery and the left subclavian artery. It compressed and stretched the left common carotid artery, compressed the pulmonary trunk and ...

  5. 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.

  6. Aortic Dissection and Postpartum Cardiomyopathy in a Postpartum Young Woman: A Case Report Study

    OpenAIRE

    Jalalian, Rozita; Saravi, Mehrdad; Banasaz, Bahar

    2014-01-01

    Introduction: Aortic dissection is a rare condition in young women and usually related with congenital anomalies of aorta and connective tissue disorders. We reported a 34-year-old postpartum woman with aortic dissection. Case Presentation: The patient complained of respiratory distress and weakness with no abdominal pain or chest pain 20 days after delivery and had no history of hypertension during pregnancy and perinatal or prior heart disease. Postpartum cardiomyopathy and left ventricular...

  7. Infected aortic aneurysm and inflammatory aortic aneurysm. In search of an optimal differential diagnosis

    International Nuclear Information System (INIS)

    Infected aortic aneurysm and inflammatory aortic aneurysm each account for a minor fraction of the total incidence of aortic aneurysm and are associated with periaortic inflammation. Despite the similarity, infected aortic aneurysm generally shows a more rapid change in clinical condition, leading to a fatal outcome; in addition, delayed diagnosis and misuse of corticosteroid or immunosuppressing drugs may lead to uncontrolled growth of microorganisms. Therefore, it is mandatory that detection of aortic aneurysm is followed by accurate differential diagnosis. In general, infected aortic aneurysm appears usually as a saccular form aneurysm with nodularity, irregular configuration; however, the differential diagnosis may not be easy sometimes for the following reasons: symptoms, such as abdominal and/or back pain and fever, and blood test abnormalities, such as elevated C-reactive protein and enhanced erythrocyte sedimentation rate, are common in infected aortic aneurysm, but they are not found infrequently in inflammatory aortic aneurysm; some inflammatory aortic aneurysms are immunoglobulin (Ig) G4-related, but not all of them; the prevalence of IgG4 positivity in infected aortic aneurysm has not been well investigated; enhanced uptake of 18F-fluorodeoxyglucose (FDG) by 18F-FDG-positron emission tomography may not distinguish between inflammation mediated by autoimmunity and that mediated by microorganism infection. Here we discuss the characteristics of these two forms of aortic aneurysm and the points of which we have to be aware before reaching a final diagnosis. (author)

  8. 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

  9. [Unicuspid Aortic Valve Stenosis Combined with Aortic Coarctation;Report of a Case].

    Science.gov (United States)

    Kubota, Takehiro; Wakasa, Satoru; Shingu, Yasushige; Matsui, Yoshiro

    2016-06-01

    Unicuspid aortic valve in an adult is extremely rare. In addition, 90% of the patients with aortic coarctation are reported to die before the age 50. A 60-year-old woman was admitted to our hospital for further examination of exertional dyspnea which had begun one year before. She had been under medical treatment for hypertension since early thirties, and had been also diagnosed with moderate aortic stenosis at 50 years of age. She was at 1st diagnosed with aortic coarctation combined with bicuspid aortic valve stenosis. The aortic valve was then found unicuspid and was replaced under cardiopulmonary bypass with perfusion to both the ascending aorta and the femoral artery. Repair of aortic coarctation was performed 3 months later through left thoracotomy without extracorporeal circulation due to the rich collateral circulation. She had no postoperative complications, and hypertension as well as ankle-brachial index improved to the normal levels. PMID:27246132

  10. 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)

  11. 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

  12. 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.)

  13. 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

  14. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... be done -- this takes a lot of real skill with your hands and your hand/eye coordination. ... we’re coming near the end of our presentation today. Dr. Rua, as you can see, is ...

  15. Aortic Aneurysm Repair

    Medline Plus

    Full Text Available ... re coming near the end of our presentation today. Dr. Rua, as you can see, is putting some stitches in. I’d like to thank my partner, Dr. Jim Benenati, who shared so much ...

  16. 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

  17. 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.

  18. Serum levels of matrix metalloproteinases -1,-2,-3 and -9 in thoracic aortic diseases and acute myocardial ischemia

    Directory of Open Access Journals (Sweden)

    Argiriadou Helena

    2009-11-01

    Full Text Available Abstract Background Matrix metalloproteinases (MMPs constitute a family of zinc-dependent proteases (endopeptidases whose catalytic action is the degradation of the extracellular matrix components. In addition, they play the major role in the degradation of collagen and in the process of tissue remodeling. The present clinical study investigated blood serum levels of metalloproteinases- 1, -2, -3 and -9 in patients with acute and chronic aortic dissection, thoracic aortic aneurysm and acute myocardial ischemia compared to healthy individuals. Methods The blood serum levels of MMP-1, -2, -3 and -9 were calculated in 31 patients with acute aortic dissection, 18 patients with chronic aortic dissection, 18 patients with aortic aneurysm and in 13 patients with acute myocardial ischemia, as well as in 15 healthy individuals who served as the control group. Serum MMP levels were measured by using an ELISA technique. Results There were significantly higher levels of MMP-3 in patients with acute myocardial ischemia as compared to acute aortic dissection (17.33 ± 2.03 ng/ml versus 12.92 ± 1.01 ng/ml, p Conclusion Measurement of serum MMP levels in thoracic aortic disease and acute myocardial ischemia is a simple and relatively rapid laboratory test that could be used as a biochemical indicator of aortic disease or acute myocardial ischemia, when evaluated in combination with imaging techniques.

  19. 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.

  20. 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.

  1. 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

  2. Delayed endovascular treatment of descending aorta stent graft collapse in a patient treated for post- traumatic aortic rupture: a case report

    OpenAIRE

    Stegher Silvia; Occhiuto Maria; Malacrida Giovanni; Mazzaccaro Daniela; Nano Giovanni; Tealdi Domenico G

    2011-01-01

    Abstract Background We report a case of delayed endovascular correction of graft collapse occurred after emergent Thoracic Endovascular Aortic Repair (TEVAR) for traumatic aortic isthmus rupture. Case presentation In 7th post-operative day after emergent TEVAR for traumatic aortic isthmus rupture (Gore TAG® 28-150), a partial collapse of the endoprosthesis at the descending tract occurred, with no signs of visceral ischemia. Considering patient's clinical conditions, the graft collapse wasn't...

  3. A case of an aortic dissection in a young adult: a refresher of the literature of this "great masquerader"

    OpenAIRE

    Vallée, Michel

    2011-01-01

    Jérôme Pineault1, Denis Ouimet2, Vincent Pichette2, Michel Vallée21Department of Internal Medicine, 2Department of Nephrology, Maisonneuve-Rosemont Hospital, Montreal, CanadaAbstract: Aortic dissection is often misdiagnosed, especially among young patients, and it is associated with a high mortality rate. We present here a case of fatal acute aortic dissection in a young man who was misdiagnosed with pericarditis. We reviewed the literature of acute aortic diss...

  4. 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

  5. Transcatheter Aortic Valve Replacement in Bicuspid Aortic Valve Disease

    DEFF Research Database (Denmark)

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

    2014-01-01

    .7%; type 1 BAV was 68.3%; and type 2 BAV was 5.0%. Multislice computed tomography (MSCT)-based TAV sizing was used in 63.5% of patients (77.1% balloon-expandable THV vs. 56.0% self-expandable THV, p = 0.02). Procedural mortality was 3.6%, with TAV embolization in 2.2% and conversion to surgery in 2.2%. The......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) or...

  6. 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...

  7. Thyroid Hypoplasia as a Cause of Congenital Hypothyroidism in Monozygotic Twins Concordant for Rubinstein-Taybi Syndrome.

    OpenAIRE

    Akın, Mustafa Ali; Güneş, Tamer; Akın, Leyla; Çoban, Dilek; Kara Oncu, Sena; Kiraz, Aslıhan; Kurtoğlu, Selim

    2011-01-01

    Rubinstein-Taybi syndrome (RSTS), a genetic disorder characterized by growth retardation, mental deficiency, dysmorphic face, broad thumbs and large toes, generally affects monozygotic twins concordantly. Thyroid hypoplasia (TH) is a common cause of congenital hypothyroidism (CH) and often accompanies dysmorphic syndromes. A pair of female twins were admitted to our neonatology unit 16 hours after delivery. They were born at 35 weeks of gestation. Both twins had an unusual dysmorphic facial a...

  8. Revisiting the craniosynostosis‐radial ray hypoplasia association: Baller‐Gerold syndrome caused by mutations in the RECQL4 gene

    OpenAIRE

    Van Maldergem, L; Siitonen, H. A; Jalkh, N.; Chouery, E; Roy, M.; Delague, V; Muenke, M; Jabs, E W; J. Cai; Wang, L. L.; Plon, S E; Fourneau, C.; Kestilä, M.; Gillerot, Y; Mégarbané, A

    2005-01-01

    Baller‐Gerold syndrome (BGS) is a rare autosomal recessive condition with radial aplasia/hypoplasia and craniosynostosis (OMIM 218600). Of >20 cases reported so far, a few appear atypical and have been reassigned to other nosologic entities, including Fanconi anaemia, Roberts SC phocomelia, and Pfeiffer syndromes after demonstration of corresponding cytogenetic or molecular abnormalities. Clinical overlap between BGS, Rothmund‐Thomson syndrome (RTS), and RAPADILINO syndrome is noticeable. Bec...

  9. CT volumetry of lumbar vertebral bodies in patients with hypoplasia L5 and bilateral spondylolysis and in normal controls

    Energy Technology Data Exchange (ETDEWEB)

    Wilms, Guido E.; Demaerel, Philippe; Keyzer, Frederik de [UZ Leuven, Campus Gasthuisberg, Department of Radiology, Leuven (Belgium); Willems, Endry [ZOL, Department of Radiology, Genk (Belgium)

    2012-08-15

    To examine the feasibility and results of calculating the volume of lumbar vertebral bodies in normal patients and patients with suspected hypoplasia of L5. Lumbar multi-detector CT was performed in 38 patients with bilateral spondylolysis and hypoplasia of L5 and in 38 normal patients. Lumbar vertebral body volume of L3, L4 and L5 was measured by CT volumetry with a semi-automated program, created with MeVisLab. In the control group, the average vertebral body volume (in cubic centimeters) of L3 was 35.93 ({+-}7.33), 36.34 ({+-}7.13) for L4 and 34.63 ({+-}6.88) for L5. In patients with suspected hypoplasia L5 the average body volume (in cubic centimeters) of L3 was 36.85 ({+-}7.37), 36.90 ({+-}6.99) for L4 and 33.14 ({+-}6.57) for L5. The difference in mean vertebral body volume for L3, L4 and L5 between both groups was statistically not significant. However, there was a statistically significant difference of the ratio L5/L4 (P < 0.001) between both groups: the mean ratio L5/L4 in the control group was 95.3 {+-} 3.9%, the ratio for the hypoplastic L5 group was 89.9 {+-} 6.3%. There was no significant difference in the vertebral body volume for L3, L4 and L5 between both groups due to inter-patient variability. However, the relation between the body volume of L5 and L4 is significantly different between both groups. The volume of the vertebral body of L5 proved to be on average 10.2% smaller than the volume of L4 in the group with hypoplasia L5 versus 4.7% in the control group. (orig.)

  10. 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

  11. 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...

  12. Perceval S aortic valve implantation in an achondroplastic Dwarf.

    Science.gov (United States)

    Baikoussis, Nikolaos G; Argiriou, Michalis; Argiriou, Orestis; Dedeilias, Panagiotis

    2016-01-01

    Despite cardiovascular disease in patients with dwarfism is not rare; there is a lack of reports referring to cardiac interventions in such patients. Dwarfism may be due to achondroplasia or hormonal growth disorders. We present a 58-year-old woman with episodes of dyspnea for several months. She underwent on transthoracic echocardiography, and she diagnosed with severe aortic valve stenosis. She referred to our department for surgical treatment of this finding. In accordance of her anthropometric characteristics and her very small aortic annulus, we had the dilemma of prosthesis selection. We decided to implant a stentless valve to optimize her effective orifice area. Our aim is to present the successful Perceval S valve implantation and the descriptions of the problems coming across in operating on these special patients. To our knowledge, this is the first case patient in which a Perceval S valve is implanted according to the international bibliography. PMID:26750695

  13. Perceval S aortic valve implantation in an achondroplastic Dwarf

    Directory of Open Access Journals (Sweden)

    Nikolaos G Baikoussis

    2016-01-01

    Full Text Available Despite cardiovascular disease in patients with dwarfism is not rare; there is a lack of reports referring to cardiac interventions in such patients. Dwarfism may be due to achondroplasia or hormonal growth disorders. We present a 58-year-old woman with episodes of dyspnea for several months. She underwent on transthoracic echocardiography, and she diagnosed with severe aortic valve stenosis. She referred to our department for surgical treatment of this finding. In accordance of her anthropometric characteristics and her very small aortic annulus, we had the dilemma of prosthesis selection. We decided to implant a stentless valve to optimize her effective orifice area. Our aim is to present the successful Perceval S valve implantation and the descriptions of the problems coming across in operating on these special patients. To our knowledge, this is the first case patient in which a Perceval S valve is implanted according to the international bibliography.

  14. 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

  15. 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

  16. 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.

  17. 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%

  18. Detection of increased frequency of thyroid hypoplasia in subjects irradiated in utero as the results of Chernobyl catastrophe

    International Nuclear Information System (INIS)

    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%

  19. 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.

  20. Clinical and immunologic outcome of patients with cartilage hair hypoplasia after hematopoietic stem cell transplantation.

    Science.gov (United States)

    Bordon, Victoria; Gennery, Andrew R; Slatter, Mary A; Vandecruys, Els; Laureys, Genevieve; Veys, Paul; Qasim, Waseem; Waseem, Qasim; Friedrich, Wilhelm; Wulfraat, Nico M; Scherer, Franziska; Cant, Andrew J; Fischer, Alain; Cavazzana-Calvo, Marina; Cavazanna-Calvo, Marina; Bredius, Robbert G M; Notarangelo, Luigi D; Mazzolari, Evelina; Neven, Benedicte; Güngör, Tayfun; Tayfun, Güngör

    2010-07-01

    Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disease caused by mutations in the RMRP gene. Beside dwarfism, CHH has a wide spectrum of clinical manifestations including variable grades of combined immunodeficiency, autoimmune complications, and malignancies. Previous reports in single CHH patients with significant immunodeficiencies have demonstrated that allogeneic hematopoietic stem cell transplantation (HSCT) is an effective treatment for the severe immunodeficiency, while growth failure remains unaffected. Because long-term experience in larger cohorts of CHH patients after HSCT is currently unreported, we performed a European collaborative survey reporting on 16 patients with CHH and immunodeficiency who underwent HSCT. Immune dysregulation, lymphoid malignancy, and autoimmunity were important features in this cohort. Thirteen patients were transplanted in early childhood ( approximately 2.5 years). The other 3 patients were transplanted at adolescent age. Of 16 patients, 10 (62.5%) were long-term survivors, with a median follow-up of 7 years. T-lymphocyte numbers and function have normalized, and autoimmunity has resolved in all survivors. HSCT should be considered in CHH patients with severe immunodeficiency/autoimmunity, before the development of severe infections, major organ damage, or malignancy might jeopardize the outcome of HSCT and the quality of life in these patients. PMID:20375313

  1. Transapical transcatheter aortic valve replacement in patients with cardiogenic shock

    OpenAIRE

    D'Ancona, Giuseppe; Pasic, Miralem; Buz, Semih; Drews, Thorsten; Dreysse, Stephan; Kukucka, Marian; Hetzer, Roland; Unbehaun, Axel

    2012-01-01

    Transcatheter aortic valve implantation (TAVI) has been introduced to treat patients at high risk for conventional surgery; however, cardiogenic shock is considered a contraindication for TAVI. The aim of the present study was to evaluate early and intermediate mortality of patients in cardiogenic shock undergoing TAVI as a rescue procedure. Patients in cardiogenic shock underwent transapical TAVI with Edwards SAPIEN (Edwards Lifesciences, Irvine, CA, USA) prosthetic valves. Preoperative, per...

  2. Acute Aortic Dissection in Third Trimester Pregnancy without Risk Factors

    OpenAIRE

    Kinney-Ham, Lisa; Nguyen, H Bryant; Steele, Robert; Walters, Elizabeth L

    2011-01-01

    Spontaneous aortic dissection in pregnancy is rare and life threatening for both the mother and the fetus. Most commonly, it is associated with connective tissue disorders, cardiac valve variants, or trauma. We present the case of a 23-year-old previously healthy woman, 36 weeks pregnant with a syncopal episode after dyspnea and vomiting. She subsequently developed cardiac arrest and underwent aggressive resuscitation, emergent thoracotomy, and cesarean delivery without recovery. On autopsy, ...

  3. 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.

  4. 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.

  5. 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

  6. An alternate solution for the treatment of ascending aortic aneurysms: the wrapping technique

    Directory of Open Access Journals (Sweden)

    Stamoulis Konstantinos

    2010-11-01

    Full Text Available Abstract Background The aortic Dacron wrapping technique is a surgical technique used under certain circumstances in cases of ascending aorta dilatation. Herein, we are presenting our experience on the method performed on multimorbid patients who denied major aortic surgery. Methods We included in our series 7 patients (5 male-2 female with mild to moderate ascending aortic dilatation, who were operated with the wrapping technique. One patient was submitted to biological aortic valve replacement during the same procedure. The number of conventionally operated patients during the same period (2 years was 21. Results Mortality during the 18-months follow-up control was 0%. One patient had to be operated with biological aortic valve replacement 18 months after the initial wrapping operation, although the diameter of her ascending aorta remained stable. Conclusions The Dacron wrapping technique is a method that can alternatively be used in multimorbid patients with mild to moderate ascending aortic dilatation without dissecting elements and has generally good results.

  7. Aortic root, not valve, calcification correlates with coronary artery calcification in patients with severe aortic stenosis

    DEFF Research Database (Denmark)

    Henein, Michael; Hällgren, Peter; Holmgren, Anders; Sörensen, Karen; Ibrahimi, Pranvera; Kofoed, Klaus Fuglsang; Larsen, Linnea Hornbech; Hassager, Christian

    2015-01-01

    calcification (AVC), due to tissue similarity between the two types of vessel rather than with the valve leaflet tissue. MATERIAL AND METHODS: We studied 212 consecutive patients (age 72.5 ± 7.9 years, 91 females) with AS requiring aortic valve replacement (AVR) in two Heart Centers, who underwent multidetector...... cardiac CT preoperatively. CAC, AVC and ARC were quantified using Agatston scoring. Correlations were tested by Spearman's test and Mann-Whitney U-test was used for comparing different subgroups; bicuspid (BAV) vs tricuspid (TAV) aortic valve. RESULTS: CAC was present in 92%, AVC in 100% and ARC in 82% of...... patients. CAC correlated with ARC (rho = 0.51, p < 0.001) but not with AVC. The number of calcified coronary arteries correlated with ARC (rho = 0.45, p < 0.001) but not with AVC. 29/152 patients had echocardiographic evidence of BAV and 123 TAV, who were older (p < 0.001) but CAC was associated with TAV...

  8. Aortic reconstruction with bovine pericardial grafts

    Directory of Open Access Journals (Sweden)

    Silveira Lindemberg Mota

    2003-01-01

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

  9. First transcatheter aortic valve implantation for severe pure aortic regurgitation in Asia

    OpenAIRE

    Chiam, Paul Toon-Lim; Ewe, See Hooi; Chua, Yeow Leng; Lim, Yean Teng

    2014-01-01

    Transcatheter aortic valve implantation (TAVI) has become the standard of care for inoperable patients with symptomatic severe aortic stenosis (AS), and an alternative to open aortic valve replacement for patients at high surgical risk. TAVI has also been performed in several groups of patients with off-label indications such as severe bicuspid AS, and as a valve-in-valve therapy for a degenerated surgical bioprosthesis. Although TAVI with CoreValve® prosthesis is technically challenging, and...

  10. 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...

  11. Gerbode Defect as a Result of Fungal Aortic Valve Endocarditis.

    Science.gov (United States)

    Pasrija, Chetan; Mohammed, Isa; Shats, Inna; Hong-Zohlman, Susie; Reoma, Junewai; Mazzeffi, Michael A; Rajagopal, Keshava

    2015-05-01

    A 63-year-old male patient with HIV disease presented with dyspnea and complete heart block, and was found to have aortic valve (AV) endocarditis secondary to Candida parapsilosis infection. Echocardiography demonstrated AV endocarditis and possible aortic root versus subannular abscess with moderate AV regurgitation (AR), a ventricular septal defect (VSD) and possible left ventricular to right atrial shunt (Gerbode defect). Large AV vegetations, subannular abscess with an acquired membranous VSD, Gerbode defect, and tricuspid annular abscess at the insertion of septal leaflet were noted intraoperatively. The patient underwent AV replacement with a stented bioprosthesis, two-sided VSD patch closure, and tricuspid valve (TV) repair with an annuloplasty ring. The left-sided patch closed the VSD and facilitated AV replacement, while the right-sided patch facilitated the TV repair. PMID:26901898

  12. 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

  13. Emergency Transcatheter Aortic Valve Implantation for Acute and Early Failure of Sutureless Perceval Aortic Valve.

    Science.gov (United States)

    Durand, Eric; Tron, Christophe; Eltchaninoff, Hélène

    2015-09-01

    We report the case of a 78-year-old woman admitted for cardiogenic shock related to acute and early failure (severe aortic regurgitation) of a Perceval sutureless aortic bioprosthesis (Sorin Group, Saluggia, Italy). Clinical stability was achieved using rescue transfemoral transcatheter aortic valve-in-valve implantation with an Edwards SAPIEN 3 prosthesis (Edwards Lifesciences, Irvine, CA). To our knowledge, we report herein the first case of successful valve-in-valve implantation using a SAPIEN 3 transcatheter heart valve in a sutureless bioprosthetic aortic valve with acute and early deterioration. PMID:26095935

  14. Left ventricular diastolic function in valvular aortic stenosis after aortic valve replacement

    Directory of Open Access Journals (Sweden)

    Ristić-Anđelkov Anđelka

    2002-01-01

    Full Text Available In adults with significant sympthomatic aortic valve stenosis, aortic valve replacement is therapy of choice. Replacement of the diseased aortic valve with a prosthetic valve yields relief of left ventricular outflow obstruction. Myocardial remodeling with regression of mass transpires as the heart adapts to the new level of after load. In patients with moderate left ventricular hypertrophy improvement in diastolic function during the first year after aortic valve replacement is visible, while in patients with extreme myocardial hypertrophic changes it was slower.

  15. The role of aortic wall CT attenuation measurements for the diagnosis of acute aortic syndromes

    International Nuclear Information System (INIS)

    Objectives: To determine if measurements of aortic wall attenuation can improve the CT diagnosis of acute aortic syndromes. Methods: CT reports from a ten year period were searched for acute aortic syndromes (AAS). Studies with both an unenhanced and a contrast enhanced (CTA) series that had resulted in the diagnosis of intramural hematoma (IMH) were reviewed. Diagnoses were confirmed by medical records. The attenuation of aortic wall abnormalities was measured. The observed attenuation threshold was validated using studies from 39 new subjects with a variety of aortic conditions. Results: The term “aortic dissection” was identified in 1206, and IMH in 124 patients’ reports. IMH was confirmed in 31 patients, 21 of whom had both unenhanced and contrast enhanced images. All 21 had pathologic CTA findings, and no CTA with IMH was normal. Attenuation of the aortic wall was greater than 45 HUs on the CTA images in all patients with IMH. When this threshold was applied to the new group, sensitivity for diagnosing AAS was 100% (19/19), and specificity 94% (16/17). Addition of unenhanced images did not improve accuracy. Conclusions: Measurements of aortic wall attenuation in CTA have a high negative predictive value for the diagnosis of acute aortic syndromes

  16. Hereditary renal adysplasia, pulmonary hypoplasia and Mayer-Rokitansky-Küster-Hauser (MRKH syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Acién Maribel

    2010-04-01

    Full Text Available Abstract Background Hereditary renal adysplasia is an autosomal dominant trait with incomplete penetrance and variable expression that is usually associated with malformative combinations (including Müllerian anomalies affecting different mesodermal organs such as the heart, lung, and urogenital system. Case report A case showing pulmonary hypoplasia, hip dysplasia, hereditary renal adysplasia, and Mayer-Rokitansky-Kuster-Hauser syndrome in adulthood is reported here. The i.v. pyelography showed right renal agenesis with a normal left kidney and ureter. Ultrasound and Magnetic Resonance Imaging also showed right renal agenesis with multicystic embryonary remnants in the right hemipelvis probably corresponding to a dysgenetic kidney. An uretrocystoscopy showed absence of ectopic ureter and of the right hemitrigone. She was scheduled for a diagnostic laparoscopy and creation of a neovagina according to the McIndoe technique with a prosthesis and skin graft. Laparoscopy confirmed the absence of the uterus. On both sides, an elongated, solid, rudimentary uterine horn could be observed. Both ovaries were also elongated, located high in both abdominal flanks and somewhat dysgenetics. A conventional cytogenetic study revealed a normal female karyotype 46, XX at a level of 550 GTG bands. A CGH analysis was performed using a 244K oligoarray CGH detecting 11 copy number variants described as normal variants in the databases. The 17q12 and 22q11.21 microdeletions described in other MRKH patients were not present in this case. Four years after operation her evolution is normal, without symptoms and the neovagina is adequately functional. The geneticists have studied her family history and the pedigree of the family is shown. Conclusions We suggest that primary damage to the mesoderm (paraaxil, intermediate, and lateral caused by mutations in a yet unidentified gene is responsible for: 1 skeletal dysplasia, 2 inappropriate interactions between the bronchial

  17. A case of an aortic dissection in a young adult: a refresher of the literature of this "great masquerader"

    Directory of Open Access Journals (Sweden)

    Pineault J

    2011-12-01

    Full Text Available Jérôme Pineault1, Denis Ouimet2, Vincent Pichette2, Michel Vallée21Department of Internal Medicine, 2Department of Nephrology, Maisonneuve-Rosemont Hospital, Montreal, CanadaAbstract: Aortic dissection is often misdiagnosed, especially among young patients, and it is associated with a high mortality rate. We present here a case of fatal acute aortic dissection in a young man who was misdiagnosed with pericarditis. We reviewed the literature of acute aortic dissection in young people and we focused particularly on clinical presentations, outcomes and investigations of aortic dissection. We report a case of a 33-year-old man with a history of uncontrolled hypertension with acute pleuretic chest pain who was transferred to our hospital for suspected pulmonary embolism and died of acute hemorragic pericardial effusion from an ascendant aortic dissection. We should never rule out aortic dissection off our differential diagnosis on the sole basis of a patient's young age.Keywords: pleuritic chest pain, hypertension, aortic dissection

  18. Congenital anomalies of aortic arch: CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Yung; Kim, Yang Min; Kim, Tae Hoon; Kim, Mi Young [Sejong General hospial, Seoul (Korea, Republic of); Lee, Jae Young [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of); Kim, Hyung Seok [Cheju Medical Center, Cheju (Korea, Republic of)

    2001-01-01

    Aortic arch anomalies result from the failure of an embryonic vascular structure to persists and regress in the usual manner during formation of the aortic arch. The anomalous aortic arch may encircle and compress the trachea and esophagus as a form of a vascular ring. The diagnosis of aortic arch anomaly and the recognition of airway compression are important because they are conditions which complicate the natural and surgical course of related diseases. CT can demonstrate the nature of anatomic structures such as thr treachea and esophagus not revealed by angiogrphy, simultaneosuly disclosing the relationship of stenotic airways and offending mediastinal vessels. Volumetric data acquisition by means of spiral CT enables three dimensional reconstruction, which can provide easy global understanding for the complex anatomy and spatial relationship of airway and cardiovascular structures. Three dimensional imaging is very useful for the physician and surgeon who are not accustomed to mentally reconstructing axial images, and can facilitate surgical planning.

  19. 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.

  20. 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....

  1. 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 ...

  2. 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...

  3. 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.

  4. 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

  5. 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.

  6. A case of megadolichobasilar anomaly complicated with abdominal aortic aneurysm

    International Nuclear Information System (INIS)

    A 41 year-old hypertensive male was admitted because of progressing left hemiparesis and dysarthria. CT demonstrated hyperdense mass with partial contast enhancement, extending from the level of lower pons to that of suprasellar cistern. Reconstructed imaging of CT showed a huge mass lesion, in which a wide curvilinear hyperdensity was demonstrated by contrast enhancement. Cerebral angiography revealed markedly elongated and dilated basilar and carotid arteries. From these findings, the prepontine hyperdense mass lesion was diagnosed as megadolichobasilar anomaly with marked wall thickening. Findings of abdominal aortic angiography and abdominal CT suggested the presence of marked atherosclerosis and abdominal aortic aneurysm with mural thrombi. Six months after initial admission, neurological symptoms gradually deteriorated and CT showed dilatation of the 3rd and lateral ventricles, suggesting the development of hydrocephalus due to compression of the aqueduct by the megadolichobasilar anomaly. Magnetic resonance imaging at this time demonstrated more details of the lesion and the deformity of the brain stem, which was not detected by conventional CT. Complications of vascular anomalies other than intracranial vasculature, such as aortic aneurysm, have also been repoted. After the introduction of CT, demonstration of a long, wide, curvilinear structure with abnormal density in the prepontine region has been reported to be diagnostic for the megadolichobasilar anomaly. This patient has had hypertension for 10 years, which probably due to chronic nephritis. He had no definite findings for angitis, but had abdominal aortic aneurysm with mural thrombi. From these findings, atherosclerosis of large vessels may have played one of the roles in the pathogenesis of this anomaly in the present case. (J.P.N.)

  7. 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

  8. [Sudden deaths due to non-traumatic aortic aneurysms rupture].

    Science.gov (United States)

    Bury, Anna; Meissner, Ewa; Szram, Stefan; Berent, Jarosław

    2011-01-01

    In this work we review two cases of ruptured aortic aneurysms which arose from congenital abnormalities of the aortic wall structure. In the first case, a 16-year old, previously untreated boy died, with no previous symptoms of an aortic aneurysm. The boy was suspected of taking drugs and even of committing suicide. A young couple found the boy's body in the wood close to the bus stop. There were no signs of violence on the corpse and the body was fully and properly dressed. The autopsy revealed enlarged (true aneurysm) and ruptured ascending aorta with about 700 ml of blood in the pericardial sac. Toxicological examination was negative. Histopathology showed abnormalities in the structure of the wall of aorta in the place of the rupture. All other body organs and vessels seemed to be normal and properly developed except the thoracic aorta, and no other morphologic abnormalities were present. In the second case, the corpse of a 30-year-old man was found in his apartment (he lived with his parents). The parents claimed he did not use drugs or alcohol. The autopsy, as in the previous case, revealed a ruptured true aneurysm of the ascending aorta with 370 g of blood in the pericardial sac. The concaved thoracic cavity was also observed. After the autopsy, the man's parents reported that in childhood, their son was diagnosed to suffer from Marfan syndrome. PMID:22715682

  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. 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

  11. Distortion of the CoreValve during transcatheter aortic valve-in-valve implantation due to valve dislocation

    International Nuclear Information System (INIS)

    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

  12. Apicoaortic Valve Conduit for a Patient with Aortic Valve Stenosis and Patent Coronary Bypass Grafts Using Cardiopulmonary Bypass.

    Science.gov (United States)

    Shackelford, Anthony G; Relle, Margaret A; Lombardi, Sarah A

    2015-12-01

    In adults over 65 years of age, aortic valve stenosis has been found to be present in 2-9% within this group. Furthermore, aortic valve replacements in patients whom have had a previous coronary artery bypass grafting surgery have a mortality rate as high as 18%. A non-conventional effective surgical approach of bypassing the aortic valve by inserting an apicoaortic valve conduit (AVC) connecting the left ventricular apex to the descending thoracic aorta has been previously documented. We describe the case of a successful implantation of an AVC in a 64-year-old Caucasian male using cardiopulmonary bypass. PMID:26834287

  13. 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.)

  14. 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.

  15. 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

  16. 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.

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

    DEFF Research Database (Denmark)

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

    2015-01-01

    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. Thoraci...

  18. 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. 

  19. New frontiers in aortic therapy: focus on current trials and devices in transcatheter aortic valve replacement.

    Science.gov (United States)

    Gutsche, Jacob T; Patel, Prakash A; Walsh, Elizabeth K; Sophocles, Aris; Chern, Sy-Yeu S; Jones, David B; Anwaruddin, Saif; Desai, Nimesh D; Weiss, Stuart J; Augoustides, John G T

    2015-04-01

    The first decade of clinical experience with transcatheter aortic valve replacement since 2002 saw the development of 2 main valve systems, namely the Edwards Sapien balloon-expandable valve series and the Medtronic self-expanding CoreValve. These 2 valve platforms now have achieved commercial approval and application worldwide in patients with severe aortic stenosis whose perioperative risk for surgical intervention is high or extreme. In the second decade of transcatheter aortic valve replacement, clinical experience and refinements in valve design have resulted in clinical drift towards lower patient risk cohorts. There are currently 2 major trials, PARTNER II and SURTAVI, that are both evaluating the role of transcatheter aortic valve replacement in intermediate-risk patient cohorts. The results from these landmark trials may usher in a new clinical paradigm for transcatheter aortic valve replacement in its second decade. PMID:25572322

  20. 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

    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......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...... 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...

  1. 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.

  2. Cine MRI of the ascending aorta in the elderly with respect to the flow signal void and aortic valve morphology

    International Nuclear Information System (INIS)

    Cine flow MRI was performed on a 1.5 Tesla system to observe signal intensity of blood flow within the ascending aorta in the elderly who had no aortic stenosis and to determine frequency of the flow signal void. Coronal and sagittal imaging planes of the ascending aorta were obtained in 27 aged patients with no known cardiac diseases (14 men and 13 women, mean age of 76) and 7 young volunteers (7 men, mean age of 24), utilizing ECG-gating, GRASS (gradient-recalled acquisition in steady state), and a flow compensation sequence. The young volunteers presented little or no signal void within the ascending aorta. In 26 (96%) of the 27 aged patients, on the other hand, signal void was demonstrated in the blood flow distal to the aortic valve during systole. The maximum length of the signal void that was measured at 318∼632 msec after the R wave of ECG ranged from 33 to 97 mm. Conventional and Doppler echocardiography was used to evaluate motion and morphology of the aortic valve in 19 of the 27 aged patients. Eighteen of these 19 subjects had aortic signal void on cine MRI. Echocardiography showed sclerotic changes of the aortic valve (i.e., increased echogenicity of the cusps and/or commissure fusion) in 10 (53%) of the 19 subjects. The mean maximum length of the signal void in the 10 patients with aortic valve sclerosis was significantly greater than that in the 9 patients with echocardiographically normal valve (68 vs.45 mm, p<0.01). These results suggest that signal void of blood flow in the ascending aorta, which is recognized as one of the characteristic findings in patients with aortic stenosis, is not a specific feature for this disease but rather a commom one in the elderly particularly those with sclerotic changes of the aortic valve. However, the length of the signal void may distinguish between nonstenotic and stenotic aortic valves. (author)

  3. Transcatheter Aortic Valve Implantation Infective Endocarditis: Current Data and Implications on Prophylaxis and Management.

    Science.gov (United States)

    Ben-Shoshan, Jeremy; Amit, Sharon; Finkelstein, Ariel

    2016-01-01

    During the last decade, transcatheter aortic valve implantation (TAVI) has become a widespread procedure for the treatment of symptomatic severe aortic stenosis in patients with high surgical risk. In conjunction with the growing experience, the adverse outcomes of TAVI have arisen, including transcatheter aortic valve infective endocarditis (TAVIE). Although rare, TAVIE has been shown as a major etiology of transcatheter aortic valve failure and its magnitude is expected to increase as TAVI will become more frequent, and long term follow-ups will accumulate. To date, large scale TAVI cohorts have restrictively addressed TAVIE-related data and details regarding TAVIE course and management are available only in sporadic case reports, which have been recently collected and published. In this review, we present a case of TAVIE from our institution and analyze the available data regarding prevalence, clinical presentation and microbiology of TAVIE, as depicted from the current literature. We discuss TAVIE treatment and prophylaxis strategies, which are expected to gain growing attention in the years to come, as TAVI will be established as a key procedure in aortic stenosis management. PMID:26710943

  4. Dysregulation of ossification-related miRNAs in circulating osteogenic progenitor cells obtained from patients with aortic stenosis.

    Science.gov (United States)

    Takahashi, Kan; Satoh, Mamoru; Takahashi, Yuji; Osaki, Takuya; Nasu, Takahito; Tamada, Makiko; Okabayashi, Hitoshi; Nakamura, Motoyuki; Morino, Yoshihiro

    2016-07-01

    CAVD (calcific aortic valve disease) is the defining feature of AS (aortic stenosis). The present study aimed to determine whether expression of ossification-related miRNAs is related to differentiation intro COPCs (circulating osteogenic progenitor cells) in patients with CAVD. The present study included 46 patients with AS and 46 controls. Twenty-nine patients underwent surgical AVR (aortic valve replacement) and 17 underwent TAVI (transcatheter aortic valve implantation). The number of COPCs was higher in the AS group than in the controls (Pperipheral blood mononuclear cells transfected with each ossification-related miRNA showed that these miRNAs controlled levels of osteocalcin protein. In conclusion, dysregulation of ossification-related miRNAs may be related to the differentiation into COPCs and may play a significant role in the pathogenesis of CAVD. PMID:27129184

  5. 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

  6. Imaging of aortic stent-grafts and endoleaks.

    Science.gov (United States)

    Thurnher, Siegfried; Cejna, Manfred

    2002-07-01

    consensus view about postprocedural management after aortic stent-graft implantation is lacking. The authors propose performing a baseline CT angiography at discharge and a biphasic CT angiography and Duplex ultrasound scan at three months. In patients with no evidence of an endoleak, CT angiography, plain film and Duplex sonography (abdomen) should be repeated every year after endovascular repair. If an endoleak is present at follow-up, immediate appropriate treatment should be initiated. PMID:12171186

  7. The treatment of a patient suffering from a ruptured abdominal aortic aneurysm and inoperative lung tumor - case report and review of literature

    International Nuclear Information System (INIS)

    A simultaneous case of abdominal aortic aneurysm and lung cancer occurs rarely in clinical practice (fewer than 1% of all cases treated). Treating patients with a simultaneous ruptured abdominal aortic aneurysm and inoperable lung cancer still arouses a great deal of controversy throughout the world. A ruptured abdominal aortic aneurysm poses immediate danger to the patient's life. Several authors express the opinion that in case of a ruptured abdominal aortic aneurysm and inoperative lung cancer life-saving surgery should not be imdertaken, and state that the doctor shoidd let the patient die with dignity. In the following article we present the case of an 84-year-old patient who, having been diagnosed earlier with an inoperative lung tumor, underwent surgery because of a ruptured abdominal aortic aneurysm. We also present a review of literature concerning this issue and discusses its ethical and legal aspects. (authors)

  8. 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

  9. 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...

  10. 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

  11. Multimodality imaging of aortic coarctation: From the fetus to the adolescent.

    Science.gov (United States)

    Gach, P; Dabadie, A; Sorensen, C; Quarello, E; Bonello, B; Pico, H; Hugues, N; Petit, P; Gorincour, G

    2016-05-01

    Aortic coarctation is a local narrowing of the aortic lumen, which is located at the level of the isthmus in 95% of patients. Aortic coarctation accounts for 5 to 8% of all congenital heart diseases. It may have an acute presentation in the form of heart failure in the neonate or may be discovered incidentally in adult because of severe treatment-resistant hypertension. Ultrasound may reveal the presence of aortic coarctation during the antenatal period. In this situation, associated abnormalities should be investigated (including karyotype), because they influence prognosis and indicates whether or not the birth should occur in a center with pediatric cardiology expertise. Postnatally, ultrasound and chest radiography are the basic imaging work-up. Computed tomography is often the second line imaging investigation in infants and young children for whom magnetic resonance imaging fails to confirm the diagnosis. Magnetic resonance imaging with cardiac synchronization is the preferred imaging tool in the post-treatment period. Aortic coarctation may be treated surgically or by endovascular techniques. Potential complications should be searched for using ultrasound and magnetic resonance imaging. PMID:27132712

  12. 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

  13. Contemporary Role of Computational Analysis in Endovascular Treatment for Thoracic Aortic Disease.

    Science.gov (United States)

    van Bogerijen, Guido H W; Tolenaar, Jip L; Conti, Michele; Auricchio, Ferdinando; Secchi, Francesco; Sardanelli, Francesco; Moll, Frans L; van Herwaarden, Joost A; Rampoldi, Vincenzo; Trimarchi, Santi

    2013-08-01

    In the past decade, thoracic endovascular aortic repair (TEVAR) has become the primary treatment option in descending aneurysm and dissection. The clinical outcome of this minimally invasive technique is strictly related to an appropriate patient/stent graft selection, hemodynamic interactions, and operator skills. In this context, a quantitative assessment of the biomechanical stress induced in the aortic wall due to the stent graft may support the planning of the procedure. Different techniques of medical imaging, like computed tomography or magnetic resonance imaging, can be used to evaluate dynamics in the thoracic aorta. Such information can also be combined with dedicated patient-specific computer-based simulations, to provide a further insight into the biomechanical aspects. In clinical practice, computational analysis might show the development of aortic disease, such as the aortic wall segments which experience higher stress in places where rupture and dissection may occur. In aortic dissections, the intimal tear is usually located at the level of the sino-tubular junction and/or at the origin of the left subclavian artery. Besides, computational models may potentially be used preoperatively to predict stent graft behavior, virtually testing the optimal stent graft sizing, deployment, and conformability, in order to provide the best endovascular treatment. The present study reviews the current literature regarding the use of computational tools for TEVAR biomechanics, highlighting their potential clinical applications. PMID:26798690

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

    Directory of Open Access Journals (Sweden)

    Savina Mannarino

    2015-11-01

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

  15. Endoluminal stent-graft management for type B aortic dissection and descending thoracic aortic aneurysm

    International Nuclear Information System (INIS)

    Objective: To evaluate the feasibility and short-term early results of endoluminal stent-graft placement in the Stanford B type aortic dissection and the descending thoracic aortic aneurysms. Methods: From March 2003 to September 2005, a total of 31 patients underwent placement of endovascular stent-grafts ,including the descending thoracic aortic aneurysm (n=2) and Standford B type aortic dissection (n=29). All patients had hypertension, with urgent onset of progressive thoracic pain in 27. Two kinds of stent-graft were used in this series: the Talent (Medtronic) in 15 and Aegis (Microportmedicine, Shanghai) in 16 patients. Follow-up was carried out with clinical observations and CTA. Results: Stent-graft implantation for the proximal entry closure was successfully performed in all patients. The DSA of immediately post deployment of the stent-graft showed complete occlusion the proximal entry tear in 23/29 patients with dissection, and complete isolation of the aneurysm in two patients with aortic aneurysm. Endoleak was revealed in 6 patients with dissection. No major complications related to the procedure were encountered. Conclusions: The interventional placement of stent-graft for Type B aortic dissection and descending thoracic aortic aneurysm is safe with satisfactory short-term outcomes. Nonetheless, longer follow-up is highly desirable to assess its real advantages. (authors)

  16. Bicuspid aortic valve and severe aortic stenosis in a newborn exposed to carbamazapine during pregnancy.

    Science.gov (United States)

    Karataş, Zehra; Karataş, Ahmet; Özlü, Tülay; Goksugur, Sevil B; Varan, Birgül

    2014-01-01

    The use of antiepileptic drugs increases the risk of major congenital malformations during pregnancy. Here, we report an infant who had a history of in-utero carbamazepine exposure and who was born with a cardiac malformation. The infant was born at 39 weeks of gestation vaginally to an epileptic mother who had been treated with carbamazepine throughout her pregnancy. He was referred due to cardiac murmur in the second week of his life. The mother had not received folic acid supplementation. Transthoracic echocardiography revealed bicuspid aortic valve, mild aortic stenosis, patent ductus arteriosus, patent foramen ovale and the renal ultrasound revealed mild left hydronephrosis. Follow-up echocardiography performed 14 weeks later showed increased severity of aortic stenosis and percutaneous balloon aortic valvuloplasty was performed. To our knowledge, there is only one case report in the literature mentioning the association of a bicuspid aortic valve and aortic stenosis with oxcarbazepine exposure, which is a structural derivative of carbamazepine. However, there are no reports for association with carbamazepine itself. Bicuspid aorta and aortic stenosis may be among the cardiac malformations that result from the teratogenic effect of carbamazepine. PMID:25584038

  17. 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.

  18. 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.

  19. An update on complications associated with transcatheter aortic valve implantation: stroke, paravalvular leak, atrioventricular block and perforation.

    Science.gov (United States)

    Chiam, Paul T L; Ewe, See Hooi

    2013-09-01

    Transcatheter aortic valve implantation (TAVI) has become an alternative therapeutic option for patients with symptomatic severe aortic stenosis at high surgical risk and the standard of care in patients who are inoperable for open aortic valve replacement. With technological evolution and increasing experience, the procedure has become more predictable. Complications of TAVI, however, are not infrequent, and can range from minor to life-threatening events. Stroke, paravalvular leak, various forms of atrioventricular block, including the need for permanent pacemakers and aortic annular and ventricular perforation will be the focus of the present review. Other complications associated with TAVI (such as vascular injury, acute kidney injury, coronary obstruction, valve malpositioning or migration) are clinically important, but are beyond the scope of this article. Understanding the occurrence and pathophysiology of these complications may provide insights into the improvement of the transcatheter devices and techniques, and aid in extending the application of TAVI to a broader population. PMID:24020674

  20. Robotic-assisted aortic valve bypass (apicoaortic conduit) for aortic stenosis.

    Science.gov (United States)

    Gammie, James S; Lehr, Eric J; Griffith, Bartley P; Dawood, Murtaza Y; Bonatti, Johannes

    2011-08-01

    Aortic valve bypass (AVB [apicoaortic conduit]) surgery consists of the construction of a valved conduit between the left ventricular apex and the descending thoracic aorta. In our institution, AVB is routinely performed without cardiopulmonary bypass or manipulation of the ascending aorta or native aortic valve. We report the case of an 83-year-old man with severe symptomatic bioprosthetic aortic stenosis, chronic thrombocytopenia, and a patent bypass graft who underwent robotically assisted beating-heart AVB through an anterior minithoracotomy. The distal anastomosis was constructed entirely using robotic telemanipulation. Robotic assistance enables the performance of beating-heart AVB through a small incision. PMID:21801931

  1. Digital substraction angiography in aortic dissection

    International Nuclear Information System (INIS)

    Regardless of the technical progress in new noninvasive methods, aortography is still regarded as a method providing the necessary information for cases of aortic dissection, especially those requiring emergency operative management. Twenty-four DS-aortographies of patients suspected for aortic dissection are reviewed. Intraarterial DSA is less hazardous owing to the reduced flow rate and quantity of contrast medium required. The relative share of inadequate images is rather high, and conventional cineaortography cannot be invariably replaced by the digital subtracted one. However, angiographic examination in cases suspected for aortic dissection may start with intraarterial digital substraction, and provided the latter proves inconclusive, conventional cineaortography may be resorted to. 6 refs., 3 figs. (orig.)

  2. 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.

  3. Iatrogenic neonatal type B aortic dissection: comprehensive MRI-based diagnosis and follow-up

    Energy Technology Data Exchange (ETDEWEB)

    Geiger, Julia; Markl, M. [University Hospital Freiburg, Department of Diagnostic Radiology and Medical Physics, Freiburg (Germany); Stiller, B.; Arnold, R. [University Hospital Freiburg, Department of Congenital Heart Disease and Pediatric Cardiology, Freiburg (Germany); Schlensak, C. [University Hospital Freiburg, Department of Cardiovascular Surgery, Freiburg (Germany)

    2011-10-15

    Neonatal aortic dissection is rare and most frequently iatrogenic. Decision making and appropriate imaging are highly challenging for pediatric cardiologists and radiologists. We present MRI and echocardiographic findings in the follow-up at 6 months of age of a boy with a conservatively treated iatrogenic neonatal aortic dissection (type B). To evaluate the morphology of the aortic arch and descending aorta, we carried out multidirectional time-resolved three-dimensional flow-analysis and contrast-enhanced MR angiography (CE-MRA). The MRI and Doppler echocardiographic results were closely comparable. Three-dimensional visualization helped assess details of blood flow acceleration and alteration caused by the dissection, and played a key role in our deciding not to treat surgically. (orig.)

  4. A large un-ruptured abdominal aortic aneurysm causing pulmonary embolism.

    Science.gov (United States)

    Sajjad, Jahangir; Ahmed, Abubakr; Coveney, Andrew; Fulton, Gregory

    2015-01-01

    A 79 years old woman presented in a peripheral hospital with dyspnea, right-sided pleuritic chest pain and cough for 3 days. On examination, she was tachycardiac and tachypneic. She had reduced air entry bilaterally on auscultation. Computed tomography-pulmonary angiogram, performed in peripheral Hospital, confirmed the diagnosis of pulmonary embolism, and she was commenced on warfarin. Ultrasonography showed no evidence of deep venous thrombosis in legs; however, ultrasound of the abdomen revealed an aortic aneurysm. She was hemodynamically stable on transfer to vascular surgery department, and her complete clinical examination revealed a pulsatile mass in the central abdomen. Computed tomography angiogram of aorta showed 8.7-cm abdominal aortic aneurysm. Venogram performed during inferior vena cava (IVC) filter insertion showed that IVC was displaced and compressed due to this large aortic aneurysm, causing thromboembolism. An open repair of the aneurysm was performed with uneventful recovery. PMID:26205717

  5. 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

  6. 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.

  7. Peran kalsium sebagai prevensi terjadinya hipoplasia enamel (The role of calcium on enamel hypoplasia prevention

    Directory of Open Access Journals (Sweden)

    Soegeng Wahluyo

    2013-09-01

    Full Text Available Background: Fluoride is a trace element found in many natural and commonly consumed by humans in the form of fluoride salts such as Sodium Fluoride (NaF. The impacts that are most often caused by the intake of fluoride is a damage of enamel tooth/enamel hypoplasi or fluorosis. The manifestations of these effects are defects in teeth with whitish colour, brown to black colour effected on the aesthetic. So that the prevention of fluorosis is required. Purpose: The aim of this study was to analyze the effect of calcium as prevention against tooth enamel fluorosis in Wistar rats caused by exposure to fluoride through indicators of Amelogenin, Calbindin- 28kDa protein expression, the matrix of tooth enamel density and distance between ameloblast cell. Methods: This was an experimental studies, post-test only control group design. This study used three groups of rats. Group 1 (Control was induced by sterile destilled water, group 2 (treatment 1 was induced by fluoride and group 3 (treatment 2 was induced by combination of fluoride and calcium. Each induction was done through sonde for 28 days. results: The results showed that the induction of fluoride causes the increased expression of Amelogenin protein; decreased expression of Calbindin-28kDa protein; a decrease in the density of the enamel matrix and widen the distance between cells ameloblast, while the result of the combination induced by fluoride and calcium showed increased protein expression of Calbindin-28kDa and increased density of the enamel matrix. Conclusion: Calcium can be used as an alternative preventive against the occurrence of enamel hypoplasia due to exposure of fluoride in Wistar rats.latar belakang: Fluorida adalah salah satu trace element yang ada dialam dan sering dikonsumsi manusia dalam bentuk garam fluorida yaitu sodium fluoride (NaF. Paparan fluorida biasanya berkaitan dengan asupan fluorida yang dapat membahayakan enamel gigi yaitu terjadinya hipoplasia enamel atau

  8. 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.

  9. 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...

  10. 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.

  11. 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

  12. 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...

  13. 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

  14. Renal flow studies after abdominal aortic aneurysmectomy and axillo-bifemoral bypass graft: case report

    Energy Technology Data Exchange (ETDEWEB)

    LaManna, M.M.; Yussen, P.S.

    1988-03-01

    Vascular disorders affecting the kidneys are either acquired or congenital. Included in this category are common multiplicity of renal arteries, the rare arteriovenous malformation, stresses due to fibromuscular disease or atherosclerosis including abdominal aortic aneurysms, arterial thrombosis, venous thrombosis, and infarction. In contrast to the group of cystic and neoplastic kidneys where scintigraphic or pathologic are not diagnostic, scintigraphic or pathologic anatomy in vascular disease is often diagnostic by nuclear medicine techniques. The authors present an interesting case of evaluation of acute renal failure in a patient abdominal aortic aneurysmectomy and axillo-bifemoral bypass graft.

  15. Renal flow studies after abdominal aortic aneurysmectomy and axillo-bifemoral bypass graft: case report

    International Nuclear Information System (INIS)

    Vascular disorders affecting the kidneys are either acquired or congenital. Included in this category are common multiplicity of renal arteries, the rare arteriovenous malformation, stresses due to fibromuscular disease or atherosclerosis including abdominal aortic aneurysms, arterial thrombosis, venous thrombosis, and infarction. In contrast to the group of cystic and neoplastic kidneys where scintigraphic or pathologic are not diagnostic, scintigraphic or pathologic anatomy in vascular disease is often diagnostic by nuclear medicine techniques. The authors present an interesting case of evaluation of acute renal failure in a patient abdominal aortic aneurysmectomy and axillo-bifemoral bypass graft

  16. 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

  17. 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

  18. Beveled reversed elephant trunk procedure for complex aortic aneurysm.

    Science.gov (United States)

    Fujikawa, Takuya; Yamamoto, Shin; Sekine, Yuji; Oshima, Susumu; Kasai, Reo; Sasaguri, Shiro

    2016-03-01

    The reversed elephant trunk procedure uses an inverted graft for distal aortic replacement before aortic arch replacement in patients with mega aorta, to reduce the risk in the second stage. However, the conventional technique restricts the maximum diameter of the inverted graft to the aortic graft diameter. We employed a beveled reversed elephant trunk procedure to overcome the discrepancy between graft diameters in a 54-year-old woman with a severely twisted ascending aortic graft and enlarging chronic dissection of the aortic arch and descending thoracic aorta. The patient was discharged with a satisfactory repair and no neurologic deficit. PMID:25406402

  19. Cardiovascular magnetic resonance of quinticuspid aortic valve with aortic regurgitation and dilated ascending aorta

    OpenAIRE

    Zhang Zhaoqi; Zhang Lijun; Meng Yanfeng; Wang Yongmei; Yang Xiaoming

    2009-01-01

    Abstract We report a rare case of a quinticuspid aortic valve associated with regurgitation and dilation of the ascending aorta, which was diagnosed and post-surgically followed up by cardiovascular magnetic resonance and dual source computed tomography.

  20. First transcatheter aortic valve implantation for severe pure aortic regurgitation in Asia.

    Science.gov (United States)

    Chiam, Paul Toon-Lim; Ewe, See Hooi; Chua, Yeow Leng; Lim, Yean Teng

    2014-02-01

    Transcatheter aortic valve implantation (TAVI) has become the standard of care for inoperable patients with symptomatic severe aortic stenosis (AS), and an alternative to open aortic valve replacement for patients at high surgical risk. TAVI has also been performed in several groups of patients with off-label indications such as severe bicuspid AS, and as a valve-in-valve therapy for a degenerated surgical bioprosthesis. Although TAVI with CoreValve® prosthesis is technically challenging, and global experience in the procedure is limited, the procedure could be a treatment option for well-selected patients with severe pure aortic regurgitation (AR). Herein, we report Asia's first case of TAVI for severe pure AR in a patient who was at extreme surgical risk, with good clinical outcome at six months. PMID:24570320

  1. Computed tomography angiography of hybrid thoracic endovascular aortic repair of the aortic arch.

    Science.gov (United States)

    Akhtar, Nila J; Oderich, Gustavo S; Vrtiska, Terri J; Williamson, Eric E; Araoz, Philip A

    2013-05-01

    Endovascular repair of the aorta has traditionally been limited to the abdominal aorta and, more recently, the descending thoracic aorta. However, recently hybrid repairs (a combination of open surgical and endovascular repair) have made endovascular repair of the aortic arch possible. Hybrid repair of the aortic arch typically involves an open surgical debranching procedure that allows for revascularization of the aortic arch vessels and subsequent endovascular stent placement. These approaches avoid the deep hypothermic circulatory arrest required for full, open surgical repair of the aortic arch. In hybrid repairs, the stent landing zone determines which branch vessels will be covered and therefore need revascularization. This article will review the preprocedure assessment with computed tomography angiography, techniques for revascularization and postprocedure complications. PMID:23621141

  2. 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.

  3. Gender-Differences in aortic dissection

    Institute of Scientific and Technical Information of China (English)

    Yu-Jang Su; Che-Hung Liu; Yu-Hang Yeh

    2014-01-01

    Objectives:Aortic dissection is a truly emergency in daily practice, and for gender factor, we want to compare the epidemiology, biomarkers, symptoms and outcome.Methods:A retrospective review six-yearAD cases in a northernTaiwan medical center fromJanuary,1, 2005 toDecember,31,2010. by gathering data of134AD patients including gender, age, episodes of time, season, vital signs, symptoms(chest pain, chest tightness(CP/CT), abdominal pain, neurological symptoms),Stanford classifications, and outcome.Comparisons are made by gender ofAD groups.85 cases with complete data are strictly enrolled into our study.We used student t test and one wayANOVA for statistical analyses, and significance was set at aP value less than 0.05(2-tailed).Results:There are64 male and21 female enrolled into our study with the mean ± standard deviation(SD) of age is(64.1±14.0) years old.InAD patients with female gender are older than maleAD patients(71.5 vs.61.6 years old,P value<0.01).In symptoms of presentation, female AD patients have more neurologic symptoms than maleAD patients(38.1% vs.12.5%,P<0.01). FemaleAD patients have longer hospital stay and higher mortality rate than maleAD patients(16.8 vs.13.4 d;38.1% vs.18.8%,P=0.39;P=0.07).Conclusion:FemaleAD patients are ten-year older in age than male, and have more common neurologic symptoms in presentations, and femaleAD patient have2-fold mortality rate than maleAD patients.

  4. Factors influencing long-term survival after aortic valve replacement.

    Directory of Open Access Journals (Sweden)

    Shigenobu,Masaharu

    1980-06-01

    Full Text Available In the aortic stenosis group, the left ventricular (LV muscle mass index was a good parameter for predicting the prognosis. Associated mitral valve disease had no influence on long term survival after aortic valve replacement. In the aortic insufficiency group, associated mitral valve disease had a marked influence on the results of aortic valve replacement. In general, the aortic insufficiency group had less clinical improvement postoperatively than the aortic stenosis group. In the annuloaortic ectasia group, left ventricular enddiastolic pressure (LVEDP might be the predictor to the prognosis. This group had the worst prognosis, of the three groups. Early operation should be considered for patients who have no, or only mild symptoms of, aortic valve disease.

  5. Anatomics Knowledges of Aortic Root in the Transcatheter Aortic Valve Implantation.

    Directory of Open Access Journals (Sweden)

    Marcelo Cerezo

    2010-12-01

    Full Text Available 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 into two groups. Group A: Formalized cadaveric dissections with hypertrofic hearts and aortic sclerosis signs from the Chair A of Anatomy of the University of La Plata (UNLP. Group B: Transthoracic Echocardiograms realized at the San Martín Hospital of La Plata between January 2005 and December 2009. Out of 67 individuals assessed, 17 (25% had one or more contraindications for TAVI. Five on Group A (36%, and 12 on Group B (23%. The most common anatomic contraindication was the Ascending Aortic diameter less than 30mm and the aortic annulus less than 20mm, following to the RAo-TSVI Angle more than 145° (2 to Group A, and 4 to Group B and the Septal thickness more than 17mm (no one to Group A, and 3 to Group B. Only just one individual was found with a low implant left coronary artery in the Group A avoiding TAVI. According to this study, the anatomic contraindications to realize TAVI are frequent due to the diagnosis devices available at the moment. The anatomic aortic root knowledge and the aortic valve interindividual variability will allow the improvement of the manufactured devices made in the market.

  6. Marfan syndrome in children and adolescents: predictive and prognostic value of aortic root growth for screening for aortic complications

    OpenAIRE

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

    1998-01-01

    Objective—To assess and measure the diagnostic and prognostic value of individual aortic root growth in children and adolescents with Marfan syndrome.
Design—From 1983 to 1996, 250 children were screened for Marfan syndrome. Serial echocardiographic aortic root measurements of 123 children (57 Marfan, 66 control) were available for evaluation of aortic root growth. Aortic root diameters were correlated with body surface area. Based on individual growth of the aortic root a discrimination form...

  7. The ascending aortic image quality and the whole aortic radiation dose of high-pitch dual-source CT angiography

    OpenAIRE

    Liu, Ying; Xu, Jian; Jian LI; Ren, Jing; LIU, HONGTAO; Xu, Junqing; Wei, Mengqi; Hao, Yuewen; Zheng, Minwen

    2013-01-01

    Background Aortic dissection is a lift-threatening medical emergency associated with high rates of morbidity and mortality. The incidence rate of aortic dissection is estimated at 5 to 30 per 1 million people per year. The prompt and correct diagnosis of aortic dissection is critical. This study was to compare the ascending aortic image quality and the whole aortic radiation dose of high-pitch dual-source CT angiography and conventional dual-source CT angiography. Methods A total of 110 conse...

  8. Evaluation of aortic regurgitation by using PC MRI: A comparison of the accuracies at different image plane locations

    Science.gov (United States)

    Kim, Byeong-Gull; Kim, Kyung-Soo; Kim, Soon-Bae; Chung, Woon-Kwan; Cho, Jae-Hwan; Park, Yong-Soon

    2012-12-01

    The goal of this study is to determine which imaging location on phase contrast magnetic resonance imaging (PC MRI) best correlates with echocardiography to enable the severity of aortic regurgitation to be accurately evaluated by using PC MRI. The subjects were 34 patients with aortic regurgitation confirmed by echocardiography and cardiac MRI. Two velocity distribution images were obtained by positioning image planes above and below the aortic valve in the PC MRI. Using the acquired images, regurgitation fractions were calculated by calculating the average forward and reverse blood flows. The severity of aortic regurgitation was then evaluated and compared with the severity as determined by using echocardiography. When image planes were positioned above the aortic valve, the regurgitation fraction obtained by using PC MRI was 44.5 ± 18.7%, and when planes were positioned below the valve, the regurgitation fraction was 34.8 ± 15.9%. Regarding agreement with echocardiographic findings, concurrence was shown to be 50% when image planes sections were positioned above the valve and 85.3% when they were positioned below the valve. The present study shows that if image planes are positioned below the valve rather than above the valve, provides as accurate evaluation of the severity of aortic regurgitation.

  9. Aorto-right atrial fistula after ascending aortic replacement or aortic value replacement

    International Nuclear Information System (INIS)

    Objective: To evaluate the CT features of aorto-right atrial fistula after aortic valve replacement (AVR) or ascending aortic replacement. Methods: Eighty-seven patients with aortic-right atrial fistula underwent CT after operation. The CT features were retrospectively analyzed. Fistula was measured according to maximum width of the shunt. Results: Aorto-right atrial fistula was detected in 87 patients after aortic valve replacement or ascending aortic replacement by CT scan. Among them, 25 patients were diagnosed as mild aorto-right atrial fistula, 47 patients as moderate, and 15 patients as severe. Thirty-seven patients underwent follow-up CT.Among them, 10 patients with mild to moderate aorto-right atrial fistula were considered to have complete regression, 8 patients with mild aorto-right atrial fistula considered to have incomplete regression, 14 patients with mild to moderate aorto-right atrial fistula considered to have stable condition, and 5 patients with moderate aorto-right atrial fistula considered to have progression at the 3-month follow-up. Conclusion: CT is a useful tool for defining aorto-right atrial fistula after AVR or ascending aortic replacement and for evaluating it in follow-up. (authors)

  10. Ultrasonic delineation of aortic microstructure: The relative contribution of elastin and collagen to aortic elasticity

    Science.gov (United States)

    Marsh, Jon N.; Takiuchi, Shin; Lin, Shiow Jiuan; Lanza, Gregory M.; Wickline, Samuel A.

    2004-05-01

    Aortic elasticity is an important factor in hemodynamic health, and compromised aortic compliance affects not only arterial dynamics but also myocardial function. A variety of pathologic processes (e.g., diabetes, Marfan's syndrome, hypertension) can affect aortic elasticity by altering the microstructure and composition of the elastin and collagen fiber networks within the tunica media. Ultrasound tissue characterization techniques can be used to obtain direct measurements of the stiffness coefficients of aorta by measurement of the speed of sound in specific directions. In this study we sought to define the contributions of elastin and collagen to the mechanical properties of aortic media by measuring the magnitude and directional dependence of the speed of sound before and after selective isolation of either the collagen or elastin fiber matrix. Formalin-fixed porcine aortas were sectioned for insonification in the circumferential, longitudinal, or radial direction and examined using high-frequency (50 MHz) ultrasound microscopy. Isolation of the collagen or elastin fiber matrices was accomplished through treatment with NaOH or formic acid, respectively. The results suggest that elastin is the primary contributor to aortic medial stiffness in the unloaded state, and that there is relatively little anisotropy in the speed of sound or stiffness in the aortic wall.

  11. Right aortic arch: a report of two cases

    International Nuclear Information System (INIS)

    A right aortic arch (RAA) is a rare congenital abnormality not always clinically manifested. The most common symptoms include dysphagia, dyspnoea on exertion, stridor, and bronchitis, resulting from a vascular ring around the trachea or esophagus. Asymptomatic cases may be viewed in adulthood. The report presents two cases of RAA found coincidentally in adult patients diagnosed using conventional chest X- ray and computed tomography (CT). Case 1 presents an abnormal course of the aortic arch, first manifested as dyspnoea in adulthood. Case 2 presents RAA with the right subclavian artery as a separate branch associated with an abnormal course of the left subclavian artery. Asymptomatic esophageal compression was also found. The abnormality was accompanied by numerous atherosclerotic lesions in peripheral vessels. Abnormal blood flow conditions might have contributed to a faster progression of atherosclerosis. RAA may remain asymptomatic for the entire life. Symptoms are caused by compression of the surrounding structures or impaired blood flow. RAA may be associated with malformations of its branches. (author)

  12. Non-invasive measurement of aortic pressure in patients: Comparing pulse wave analysis and applanation tonometry

    Directory of Open Access Journals (Sweden)

    M.U.R. Naidu

    2012-01-01

    Full Text Available Objective: The aim of the present study was to validate and compare novel methods to determine aortic blood pressure non-invasively based on Oscillometric Pulse Wave Velocity (PWV measurement using four limb-cuff pressure waveforms and two lead Electrocardiogram (ECG with a validated tonometric pulse wave analysis system in patients. Materials and Methods: After receiving the consent, in 49 patients with hypertension, coronary artery disease, diabetes mellitus, PWV, and central blood pressures were recorded in a randomised manner using both the oscillometric and tonometric devices. All recordings were performed 10 minutes after the patient lying comfortably in a noise-free temperature-controlled room. The test was performed between 09 am and 10 am after overnight fast. A minimum of three measurements were performed by the same skilled and trained operator. From the raw data obtained with two devices, software calculated the final vascular parameters. Results: A total of 49 patients (8 women and 41 men, of mean age 40.5 years (range: 19-81 years participated in the present study. After transforming the brachial pressures into aortic pressures, the correlation coefficient between the Aortic Systolic Pressure (ASP values obtained with two methods was 0.9796 (P<0.0001. The mean difference between ASP with two methods was 0.3 mm Hg. Similarly, Aortic Diastolic Pressure (ADP values obtained with two methods also correlated significantly with correlation coefficient of 0.9769 (P<0.0001. The mean difference of ADP was 0.2 mm Hg. In case of Aortic Pulse Pressure (APP, the mean difference was 0.1 mm Hg. All parameters of central aortic pressures obtained with two methods correlated significantly. Conclusion: The new method of transforming the Carotid Femoral PWV (cfPWV and brachial blood pressure values into aortic blood pressure values seems to be reasonably good. The significant correlation between the values obtained by tonometric device and

  13. A Practical Guide to Clinical Management of Thoracic Aortic Disease.

    Science.gov (United States)

    Bradley, Timothy J; Alvarez, Nanette A M; Horne, S Gabrielle

    2016-01-01

    Physicians and surgeons faced with patients with thoracic aortic disease (TAD) need to determine the underlying diagnosis to facilitate decisions regarding appropriate investigations as well as which other specialists to involve, when to start medical therapy, when to refer for surgery, and how to plan follow-up and family screening. Increased understanding of conditions predisposing to thoracic aortic aneurysm (TAA) provides the opportunity for more personalized care. However, given advances in the genetics of TAD, clinicians are now faced with an expanded and often confusing list of associated differential diagnoses. We present a practical guide to managing patients with TAD based on current knowledge and guidelines. Important "flags" on history taking and "tips" to diagnosis on physical examination along with what investigations to order and what referrals to request are discussed. Need for medical therapy, indications for surgical repair, and planning long-term follow-up of TAD are determined by age, the underlying TAD diagnosis, previous vascular history in the patient or other family members (or both), aortic dimensions and growth rate, and any coexisting cardiovascular disease. Although medications may slow the progression of TAA, effective aortic surveillance and timely elective surgical repair remain the mainstays of prevention of acute aortic complications. Emergent repair of acute aortic dissection carries a far worse prognosis. Taking a practical approach to the management of TAD allows for standardized assessment and implementation of current best practice clinical guidelines. Ongoing discovery of new genes, better medical therapies, and innovative surgical techniques necessitate constantly adapting knowledge and integrating it into everyday clinical practice. PMID:26724515

  14. Double aortic arch as a source of airway obstruction in a child

    Directory of Open Access Journals (Sweden)

    Sambhunath Das

    2015-01-01

    Full Text Available Double aortic arch (DAA is a congenital vascular anomaly. The diagnosis was difficult till the child was symptomatic, and other causes were ruled out. We present the interesting images of a child of respiratory distress because of tracheal compression from DAA.

  15. Embolization with Histoacryl Glue of an Anastomotic Pseudoaneurysm following Surgical Repair of Abdominal Aortic Aneurysm

    Science.gov (United States)

    Walid, Ayesha; Ul Haq, Tanveer; Ur Rehman, Zia

    2013-01-01

    We report a 62-year-old female who had surgical repair of abdominal aortic aneurysm with a bifurcated graft 2 years ago. She presented with a distal anastomotic pseudoaneurysm which was successfully embolized with histoacryl glue. Only one such similar case has been reported in the literature so far (Yamagami et al. (2006)). PMID:23476883

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

    Science.gov (United States)

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

    2016-01-01

    Presented herein is a technique of unilateral antegrade perfusion of the brain in operations on the aortic arch. The method makes it possible to perform both systemic artificial circulation and adequate physiological perfusion of the brain, promoting minimization of the number of neurological complications. PMID:27100557

  17. The study of aortic stiffness in different hypertension subtypes in patients with chronic kidney disease

    Institute of Scientific and Technical Information of China (English)

    布海霞

    2014-01-01

    Objective To investigate whether there is any difference in aortic stiffness among different hypertension subtypes in patients with chronic kidney disease.Methods Six hundred and twenty-six patients with chronic kidney disease were included in the present analysis.They were classified into four groups:normotension(n=391)with systolic blood pressure(SBP)<140 mmHg and diastolic

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

    OpenAIRE

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

    1981-01-01

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

  19. Pseudoaneurysm of mitral-aortic intervalvular fibrosa in a child: Demonstration by MDCT and MRI

    International Nuclear Information System (INIS)

    Subaortic left ventricular outflow tract pseudoaneurysms are rare lesions that are associated with aortic valve diseases, infective endocarditis, trauma or surgery. We present dynamic multidetector computerized tomography and cine magnetic resonance imaging features of a case of subaortic aneurysm arising from interaortic-mitral valvular region in a child with past history of infective endocarditis

  20. Strategies to treat thoracic aortitis and infected aortic grafts.

    Science.gov (United States)

    Kahlberg, A; Melissano, G; Tshomba, Y; Leopardi, M; Chiesa, R

    2015-04-01

    Infectious thoracic aortitis is a rare disease, especially since the incidence of syphilis and tuberculosis has dropped in western countries. However, the risk to develop an infectious aortitis and subsequent mycotic aneurysm formation is still present, particularly in case of associated endocarditis, sepsis, and in immunosuppressive disorders. Moreover, the number of surgical and endovascular thoracic aortic repairs is continuously increasing, and infective graft complications are observed more frequently. Several etiopathogenetic factors may play a role in thoracic aortic and prosthetic infections, including hematogenous seeding, local bacterial translocation, and iatrogenous contamination. Also, fistulization of the esophagus or the bronchial tree is commonly associated with these diseases, and it represents a critical event requiring a multidisciplinary management. Knowledge on underlying micro-organisms, antibiotic efficacy, risk factors, and prevention strategies has a key role in the management of this spectrum of infectious diseases involving the thoracic aorta. When the diagnosis of a mycotic aneurysm or a prosthetic graft infection is established, treatment is demanding, often including a number of surgical options. Patients are usually severely compromised by sepsis, and in most cases they are considered unfit for surgery for general clinical conditions or local concerns. Thus, results of different therapeutic strategies for infectious diseases of the thoracic aorta are still burdened with very high morbidity and mortality. In this manuscript, we review the literature regarding the main issues related to thoracic infectious aortitis and aortic graft infections, and we report our personal series of patients surgically treated at our institution for these conditions from 1993 to 2014. PMID:25608572