Atypical initial presentation of Takayasu arteritis as isolated supra-valvular aortic stenosis.

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Kim, Do Yeon; Kim, Hwan Wook

2016-01-19

Among the vascular involvements of Takayasu arteritis, a supra-valvular aortic stenosis has been reported very rarely. We report a case of surgically corrected, supra-valvular aortic stenosis caused by Takayasu arteritis. A 32-year-old female was diagnosed with supra-valvular aortic stenosis by transthoracic echocardiography for the evaluation of cardiac murmur with constitutional symptoms. Under the impression of non-familial sporadic type of supra-valvular aortic stenosis, surgical correction was performed. However, after 1 year from the operation, we could know the cause of her disease through the findings of computed tomographic aortography that Takayasu arteritis was suspected. Takayasu arteritis should be considered in adult female patients presenting supra-valvular aortic stenosis with constitutional symptoms, even if no typical features of vascular involvement.

MR findings in pontocerebellar hypoplasia

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

1998-07-01

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

MR findings in pontocerebellar hypoplasia

International Nuclear Information System (INIS)

Uhl, M.; Pawlik, H.; Laubenberger, J.; Langer, M.; Darge, K.; Baborie, A.; Korinthenberg, R.

1998-01-01

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.)

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

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

2015-01-01

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

Acute Contained Ruptured Aortic Aneurysm Presenting as Left Vocal Fold Immobility

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Sharon H. Gnagi

2015-01-01

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

Vermian aplasia and hypoplasia

International Nuclear Information System (INIS)

Fitz, C.R.

1990-01-01

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

Genetics Home Reference: focal dermal hypoplasia

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... in people with focal dermal hypoplasia is an omphalocele , which is an opening in the wall of ... Dermal Hypoplasia MedlinePlus Encyclopedia: Ectodermal dysplasia MedlinePlus Encyclopedia: Omphalocele General Information from MedlinePlus (5 links) Diagnostic Tests ...

Unusual presentation of aortic dissection: Post-coital acute paraplegia with renal failure

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Dinith P Galabada

2014-01-01

Full Text Available We report the case of a 45-year-old chronic smoker who presented with acute paraplegia occurring during coitus and subsequently developed acute renal failure (ARF requiring dialysis. He had absent peripheral pulses in the lower limbs with evidence of acute ischemia. Doppler study showed dissecting aneurysm of thoracic aorta, thrombotic occlusion of the distal aorta from L1 level up to bifurcation and occlusion of the right renal artery by a thrombus that was confirmed by magnetic resonance imaging of the spine. He was not subjected to any vascular intervention as his lower limbs were not salvageable due to delay in the diagnosis. Post-coital aortic dissection and aortic dissection presenting with acute paraplegia and ARF are very rare. This is probably the first case report with post-coital acute aortic dissection presenting with paraplegia and ARF. This case emphasizes the importance of a careful examination of peripheral pulses in patients presenting with ARF and paraplegia.

MR assessment of fetal pulmonary hypoplasia

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

2000-08-01

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

Acute Contained Ruptured Aortic Aneurysm Presenting as Left Vocal Fold Immobility

OpenAIRE

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

2015-01-01

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

Pregnancy Outcome in Cartilage-Hair Hypoplasia, a Rare Form of Dwarfism

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Harshithaa Thavarajah

2017-01-01

Full Text Available Background. This case report discusses the pregnancy outcome of a patient with cartilage-hair hypoplasia, a rare form of dwarfism, and multiple previous orthopedic surgeries. Literature on pregnancy outcomes in patients with cartilage-hair hypoplasia is limited. Case. A 32-year-old patient with cartilage-hair hypoplasia presented at 12 weeks’ gestation to the high-risk obstetrics clinic for care. Preterm labor resulted in cesarean delivery at 34 weeks’ gestation with general anesthetic. Breastfeeding was stopped at 6 weeks due to neonatal complications. Conclusion. Pregnancy and delivery were uncomplicated. A multidisciplinary approach allowed for effective management during pregnancy and postnatal care. This is the first known documented case of prenatal care, delivery, and breastfeeding in a woman with this rare disorder.

Congenital Unilateral Hypoplasia of Depressor Anguli Oris

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Seckin O. Ulualp

2012-01-01

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

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

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

2014-10-01

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

Ruptured Aortic Aneurysm Presenting as a Stridor

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Feng Lin

2010-06-01

Full Text Available Stridor is an abnormal, high-pitched, whining breathing sound caused by a blockage in the throat or larynx that is usually heard in children. We describe an unusual case of an 81-year-old man brought to our emergency department with sudden onset of dyspnea and shortness of breath. Stridor could be heard without a stethoscope. We found a huge mass over the left upper chest on chest radiography, suggesting an aortic aneurysm. We believed that these symptoms were caused by a huge thoracic aortic aneurysm with trachea/bronchi compression. Chest computed tomography confirmed the diagnosis.

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

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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.

The radiological features of Goltz syndrome: Focal dermal hypoplasia

International Nuclear Information System (INIS)

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

1988-01-01

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)

Brain stem hypoplasia associated with Cri-du-Chat syndrome

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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.

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

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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.

Type A Aortic Dissection Presenting with Inferior ST-Elevation Myocardial Infarction.

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Wu, Bao-Tzung; Li, Chun-Yi; Chen, Ying-Tsung

2014-05-01

Type A aortic dissection with concurrent ST-elevation myocardial infarction (STEMI) is relatively rare. However, it can be potentially fatal and easily misdiagnosed as STEMI alone. Misdiagnosis will lead to inappropriate administration of anticoagulant and thrombolytic therapy and delayed surgical repair of the aorta. In patients with STEMI, short reperfusion time is associated with improved survival, and minimizing the door-to-balloon time is the goal of therapy worldwide. However, signs critical for differential diagnosis may be overlooked in the rush to primary percutaneous coronary intervention. When a patient is encountered who presents with chest pain and ST elevation on electrocardiogram, STEMI should not be the only diagnosis considered. By using bedside available information, detailed history taking and focused physical examination, it is possible to avoid a mistaken diagnosis. Here we report a case of Stanford type A aortic dissection with STEMI that was initially misdiagnosed as sole acute inferior wall myocardial infarction. Patient mortality may have resulted from delayed diagnosis and surgical treatment. Acute myocardial infarction; Aortic dissection.

Focal dermal hypoplasia: A rare case report

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Sahana M Srinivas

2015-01-01

Full Text Available Focal dermal hypoplasia (Goltz syndrome is a rare genetic multisystem disorder primarily involving the skin, skeletal system, eyes, and face. We report the case of an eight-month-old female child who presented with multiple hypopigmented atrophic macules along the lines of blaschko, skeletal anomalies, umbilical hernia, developmental delay, hypoplastic nails, syndactyly, and lobster claw deformity characteristic of Goltz syndrome.

Prenatal and neonatal variables associated with enamel hypoplasia in deciduous teeth in low birth weight preterm infants

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Kátia Maria Dmytraczenko Franco

2007-12-01

Full Text Available This study investigated possible prenatal and neonatal variables that may influence the prevalence of tooth enamel hypoplasia in preterm and low birth weight children (LBW and a matched control group of term children with normal birth weight (NBW. The study sample consisted of 61 children born preterm and with LBW examined at 18-34 months of age. The control group was formed by 61 infants born full term and with NBW examined at 31-35 months of age. All children were born at the Center of Integrated Attention of Women's Health (CAISM-UNICAMP. FDI criteria were followed for dental examination. Medical data was collected retrospectively from hospital records. Among preterms, 57.4% had some type of developmental defects of enamel (DDE, 52.5 % had opacities and 21.3 % presented hypoplasia. Among full-term children, 24.6% presented DDE, 24.6% had opacities and 3.3% had hypoplasia. LBW preterm infants presented a higher prevalence of hypoplasia than NBW controls. The deciduous teeth most affected by hypoplasia were maxillary incisors. There was no significant association with prenatal variables; among neonatal variables there was a significant association with respiratory distress syndrome and neurological examination at discharge with an altered result.

Alkaptonuria Presenting with Impressive Osteoarticular Changes and Severe Aortic Stenosis.

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Roca, Bernardino; Roca, Manuel; Monferrer, Raquel

2016-03-01

Alkaptonuria, or ochronosis, a rare autosomal recessive metabolic disorder, causes an excess of homogentisic acid that results in dark pigmentation, calcification, and inflammation of cartilaginous and other tissues. Cardiovascular complications are also typical of the disease. We report the case of a 78-year-old male who presented with impressive osteoarticular changes and aortic stenosis associated with alkaptonuria.

Unlikely culprit: congenital middle aortic syndrome diagnosed in the sixth decade of life.

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Ali, Muhammad Sajawal; Tchernodrinski, Stefan; Mohananey, Divyanshu; Ali, Ahya Sajawal

2016-08-16

A 58-year-old woman was admitted with acute heart failure. She had a long history of resistant hypertension, with an unremarkable work up for secondary causes in the past. Her brachial blood pressure was 210/70 mm Hg, with ankle blood pressure of 100/70 mm Hg. CT angiogram revealed marked narrowing of the descending thoracic aorta between the left subclavian artery and the diaphragm, consistent with middle aortic syndrome (MAS). She was initially managed with diuretics and antihypertensives. Subsequently thoracotomy revealed a severely hypoplastic segment of the descending aorta. The diseased segment was resected and aortic reconstruction performed. Histopathology showed fragmentation of the medial elastic fibres and fibrosis of the medial and intimal layers. These findings along with gross aortic hypoplasia and absence of features of Takayasu's arteritis, suggest that our patient had congenital MAS. The patient has done well since her surgery. We believe this is the first case of congenital MAS reported in the sixth decade of life. 2016 BMJ Publishing Group Ltd.

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

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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.

Clinical Features and Management of Cartilage-Hair Hypoplasia: A Narrative Review

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Kobra Shiasi Arani

2015-01-01

Full Text Available Context: Cartilage-hair hypoplasia is a rare hereditary cause of short stature. The aim of this study was to familiarize physicians with this rare but important disease. Evidence Acquisition: This article is a narrative review of the scientific literature to inform about clinical features and management of Cartilage-hair hypoplasia. A systematic search identified 127 papers include original and review articles and case reports. Results: Cartilage-Hair Hypoplasia characterized by short-limb dwarfism associated with metaphyseal chondrodysplasia. The inheritance is autosomal recessive. Other findings include hair hypoplasia, anemia, immunodeficiency, propensity to infections, gastrointestinal disorders (Hirschsprung disease, anal stenosis, esophageal atresia and malabsorption, defective spermatogenesis, increased risk of malignancies and higher rate of mortality. Immunodeficiency in cartilage-hair hypoplasia may be an isolated B-cell or isolated T-cell immunodeficiency or combined B and T-cell immunodeficiency; however, severe combined immunodeficiency is rare. There is no known treatment for hair hypoplasia. Growth hormone was used with conflicting results for short stature in children with Cartilage-hair hypoplasia. Skeletal problems must be managed with physiotherapy and appropriate orthopedic interventions. Hirschsprung disease, anal stenosis and esophageal atresia should be surgically corrected. Patients with severe hypoplastic anemia require repeated transfusions. Bone marrow transplantation may be required for patients with severe combined immunodeficiency or severe persistent hypoplastic anemia. Treatment with G-CSF is useful for neutropenia. Patients should be monitored closely for developing malignancy such as skin neoplasms, lymphomas and leukemias. Conclusions: Cartilage-hair hypoplasia is an important hereditary disease with different medical aspects. The high rate of consanguineous marriages in Iran necessitates considering CHH in any

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

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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

Traumatic aortic dissection presenting with respiratory arrest

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C.C. Meyer

2015-03-01

Conclusion: Blunt aortic injury is a major cause of death in blunt chest trauma. Signs and symptoms of this injury are nonspecific and other injuries often take precedence, which makes a relatively difficult diagnosis even more challenging.

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

International Nuclear Information System (INIS)

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

1995-01-01

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

Impact of Aortic Insufficiency on Ascending Aortic Dilatation and Adverse Aortic Events After Isolated Aortic Valve Replacement in Patients With a Bicuspid Aortic Valve.

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Wang, Yongshi; Wu, Boting; Li, Jun; Dong, Lili; Wang, Chunsheng; Shu, Xianhong

2016-05-01

Aberrant flow pattern and congenital fragility bestows bicuspid aortic valve (BAV) with a propensity toward ascending aorta dilatation, aneurysm, and dissection. Whether isolated aortic valve replacement (AVR) can prevent further dilatation in BAV ascending aorta and what indicates concurrent aortic intervention in the case of valve operation remain controversial. From June 2006 to January 2009, patients with a BAV who underwent isolated AVR were consecutively included and categorized into aortic insufficiency (BAV-AI, n = 84) and aortic stenosis (n = 112) groups, and another population of patients with a tricuspid aortic valve with aortic insufficiency (n = 149) was also recruited during the same period for comparison of annual aortic dilatation rate and adverse aortic events after isolated AVR. With a median follow-up period of 72 months (interquartile range, 66 to 78 months), ascending aorta dilatation rates were faster in the BAV-AI group than the BAV plus aortic stenosis and tricuspid aortic valve with aortic insufficiency groups (both p regression analysis identified aortic insufficiency (hazard ratio, 3.7; 95% confidence interval, 1.2 to 11.1; p = 0.019) as an independent risk factor for adverse aortic events among patients with BAV in general, whereas preoperative ascending aortic diameter larger than 45 mm (hazard ratio, 13.8; 95% confidence interval, 3.0 to 63.3; p = 0.001) served as a prognostic indicator in the BAV-AI group. An aggressive policy of preventive aortic interventions seemed appropriate in patients with BAV-AI during AVR, and BAV phenotype presenting as either insufficiency or stenosis should be taken into consideration when contemplating optimal surgical strategies for BAV aortopathy. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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.

Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis

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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.

Amelogenesis Imperfect, Enamel Hypoplasia and Fluorosis Dental - Literature Review

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Flávia Magnani Bevilacqua

2015-12-01

Full Text Available The developmental disorders of enamel are abnormalities of structure which can affect both dentitions. These abnormalities include amelogenesis imperfecta, enamel hypoplasia and dental fluorosis. The amelogenesis imperfecta is a hereditary change and enamel hypoplasia is a quantitative defect of enamel that occurs as a result of systemic problems, local and also inherited factors, or even the combination of them. Dental fluorosis is a hypoplasia caused by the chronic ingestion of fluoride during odontogenesis. All these anomalies have similar clinical characteristics, and it is necessary to be careful in their assessment. It is extremely important to know these abnormalities to establish a differential diagnosis and, consequently, a treatment plan, which can be set for each situation. Therefore, the purpose of this study was to review the literature regarding these three anomalies: amelogenesis imperfecta, enamel hypoplasia and dental fluorosis. It was concluded that to establish the differential diagnosis of these abnormalities as well as a proper treatment plan, it is indispensable the professional knowledge associated with the clinical examination. The examination has to consist of medical history and physical examination, and in some cases, x-ray examination.

Familial Thoracic Aortic Aneurysm with Dissection Presenting as Flash Pulmonary Edema in a 26-Year-Old Man

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Sabry Omar

2014-01-01

Full Text Available We are reporting a case of familial thoracic aortic aneurysm and dissection in a 26-year-old man with no significant past medical history and a family history of dissecting aortic aneurysm in his mother at the age of 40. The patient presented with cough, shortness of breath, and chest pain. Chest X-ray showed bilateral pulmonary infiltrates. CT scan of the chest showed a dissection of the ascending aorta. The patient underwent aortic dissection repair and three months later he returned to our hospital with new complaints of back pain. CT angiography showed a new aortic dissection extending from the left carotid artery through the bifurcation and into the iliac arteries. The patient underwent replacement of the aortic root, ascending aorta, total aortic arch, and aortic valve. The patient recovered well postoperatively. Genetic studies of the patient and his children revealed no mutations in ACTA2, TGFBR1, TGFBR2, TGFB2, MYH11, MYLK, SMAD3, or FBN1. This case report focuses on a patient with familial TAAD and discusses the associated genetic loci and available screening methods. It is important to recognize potential cases of familial TAAD and understand the available screening methods since early diagnosis allows appropriate management of risk factors and treatment when necessary.

Biliary atresia and cerebellar hypoplasia in polysplenia syndrome

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Vanderdood, Kurt; Op de Beeck, Bart; Desprechins, Brigitte; Osteaux, Michel [Department of Radiology, Free University Brussels, AZ-VUB, Laarbeeklaan 101, 1090 Brussels (Belgium)

2003-09-01

We report a 3.5-month-old boy with polysplenia syndrome who demonstrated hemiazygos continuation of the inferior vena cava, extrahepatic biliary atresia, multiple splenunculi, bowel malrotation, and the rare finding of brainstem and cerebellar hypoplasia. A possible pathogenesis for cerebellar hypoplasia in this syndrome is suggested after review of the literature. The importance of seeking associated anomalies in biliary atresia, which may be possible indicators of polysplenia syndrome, is stressed since these patients need appropriate management when surgery is considered. (orig.)

Internal Carotid Artery Hypoplasia: Role of Color-Coded Carotid Duplex Sonography.

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Chen, Pei-Ya; Liu, Hung-Yu; Lim, Kun-Eng; Lin, Shinn-Kuang

2015-10-01

The purpose of this study was to determine the role of color-coded carotid duplex sonography for diagnosis of internal carotid artery hypoplasia. We retrospectively reviewed 25,000 color-coded carotid duplex sonograms in our neurosonographic database to establish more diagnostic criteria for internal carotid artery hypoplasia. A definitive diagnosis of internal carotid artery hypoplasia was made in 9 patients. Diagnostic findings on color-coded carotid duplex imaging include a long segmental small-caliber lumen (52% diameter) with markedly decreased flow (13% flow volume) in the affected internal carotid artery relative to the contralateral side but without intraluminal lesions. Indirect findings included markedly increased total flow volume (an increase of 133%) in both vertebral arteries, antegrade ipsilateral ophthalmic arterial flow, and a reduced vessel diameter with increased flow resistance in the ipsilateral common carotid artery. Ten patients with distal internal carotid artery dissection showed a similar color-coded duplex pattern, but the reductions in the internal and common carotid artery diameters and increase in collateral flow from the vertebral artery were less prominent than those in hypoplasia. The ipsilateral ophthalmic arterial flow was retrograde in 40% of patients with distal internal carotid artery dissection. In addition, thin-section axial and sagittal computed tomograms of the skull base could show the small diameter of the carotid canal in internal carotid artery hypoplasia and help distinguish hypoplasia from distal internal carotid artery dissection. Color-coded carotid duplex sonography provides important clues for establishing a diagnosis of internal carotid artery hypoplasia. A hypoplastic carotid canal can be shown by thin-section axial and sagittal skull base computed tomography to confirm the final diagnosis. © 2015 by the American Institute of Ultrasound in Medicine.

[Pulmonary hypoplasia: An analysis of cases over a 20-year period].

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Delgado-Peña, Yanny Paola; Torrent-Vernetta, Alba; Sacoto, Gabriela; de Mir-Messa, Inés; Rovira-Amigo, Sandra; Gartner, Silvia; Moreno-Galdó, Antonio; Molino-Gahete, José Andrés; Castillo-Salinas, Felíx

2016-08-01

Pulmonary hypoplasia is the most frequent congenital anomaly associated with perinatal mortality. A retrospective and descriptive review was conducted on cases of patients diagnosed with pulmonary hypoplasia between 1995 and 2014 in a tertiary university hospital. An analysis was made of the prenatal imaging, clinical manifestations, post-natal diagnostic tests, treatment and management, long-term follow up, and survival data. A total of 60 cases were identified, all of them with prenatal imaging. Sixteen patients required foetal surgery. Congenital diaphragmatic hernia was the most frequent diagnosis. Main clinical presentation was respiratory distress with severe hypoxemia and high requirements of mechanical ventilation. Mortality rate was 47% within first 60 days of life, and 75% for the first day of life. Pneumonia and recurrent bronchitis episodes were observed during follow-up. They had a lung function obstructive pattern, and their quality of life and exercise tolerance was good. High neonatal mortality and significant long-term morbidity associated with pulmonary hypoplasia requires an early diagnosis and a specialised multidisciplinary team management. Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Use of repeat anterior maxillary distraction to correct residual midface hypoplasia in cleft patients.

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Richardson, Sunil; Krishna, Shreya; Bansal, Avi

2017-12-01

The study was designed to evaluate the efficacy of performing a second, repeat anterior maxillary distraction (AMD) to treat residual cleft maxillary hypoplasia. Five patients between the ages of 12 to 15 years with a history of AMD and with residual cleft maxillary hypoplasia were included in the study. Inclusion was irrespective of gender, type of cleft lip and palate, and the amount of advancement needed. Repeat AMD was executed in these patients 4 to 5 years after the primary AMD procedure to correct the cleft maxillary hypoplasia that had developed since the initial procedure. Orthopantomogram (OPG) and lateral cephalograms were taken for evaluation preoperatively, immediately after distraction, after consolidation, and one year postoperatively. The data obtained was tabulated and a Mann Whitney U-test was used for statistical comparisons. At the time of presentation, a residual maxillary hypoplasia was observed with a well maintained distraction gap on the OPG which ruled out the occurrence of a relapse. Favorable movement of the segments without any resistance was seen in all patients. Mean maxillary advancement of 10.56 mm was achieved at repeat AMD. Statistically significant increases in midfacial length, SNA angle, and nasion perpendicular to point A distance was achieved ( P =0.012, P =0.011, and P =0.012, respectively). Good profile was achieved for all patients. Minimal transient complications, for example anterior open bite and bleeding episodes, were managed. Addressing the problem of cleft maxillary hypoplasia at an early age (12-15 years) is beneficial for the child. Residual hypoplasia may develop in some patients, which may require additional corrective procedures. The results of our study show that AMD can be repeated when residual deformity develops with the previous procedure having no negative impact on the results of the repeat procedure.

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

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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

Aortic Annular Enlargement during Aortic Valve Replacement

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Selman Dumani

2016-09-01

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

Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

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Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong [Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang (Korea, Republic of); Kim, Seon Jeong [Dept. of Radiology, Myongji Hospital, Goyang (Korea, Republic of)

2017-05-15

Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia.

Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

International Nuclear Information System (INIS)

Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong; Kim, Seon Jeong

2017-01-01

Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia

Chronic Bilateral Slipped Capital Femoral Epiphysis as an Unusual Presentation of Congenital Panhypopituitarism due to Pituitary Hypoplasia in a 17-Year-Old Female

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Sasigarn A. Bowden

2009-01-01

Full Text Available We report an interesting case of a 17-year-old normal-statured female who was diagnosed with congenital panhypopituitarism due to pituitary hypoplasia at the presentation of bilateral slipped capital femoral epiphysis. We emphasized the importance of endocrinologic evaluation in patients with atypical slipped capital femoral epiphysis to prevent potential complication of adrenal crisis during surgery. This case also demonstrates growth without growth hormone which resulted in a delay in diagnosis of congenital hypopituitarism in this patient.

Chronic Bilateral Slipped Capital Femoral Epiphysis as an Unusual Presentation of Congenital Panhypopituitarism due to Pituitary Hypoplasia in a 17-Year-Old Female

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Klingele KevinE

2009-11-01

Full Text Available We report an interesting case of a 17-year-old normal-statured female who was diagnosed with congenital panhypopituitarism due to pituitary hypoplasia at the presentation of bilateral slipped capital femoral epiphysis. We emphasized the importance of endocrinologic evaluation in patients with atypical slipped capital femoral epiphysis to prevent potential complication of adrenal crisis during surgery. This case also demonstrates growth without growth hormone which resulted in a delay in diagnosis of congenital hypopituitarism in this patient.

Evidence of aortic dissection and Marfan syndrome in a mummy from the Capuchin Catacombs of Palermo, Sicily.

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Panzer, Stephanie; Thompson, Randall C; Hergan, Klaus; Zink, Albert R; Piombino-Mascali, Dario

2018-06-08

The authors report on the assessment of an anthropogenic mummy of a young man from the Capuchin Catacombs of Palermo, Sicily, tentatively dated from the mid- to late 19 th century AD. The mummy was investigated by full-body CT examination. CT images clearly showed aortic dissection classified as Stanford-A. Due to the relation of aortic dissection to inherited connective tissue diseases in young people, such as Marfan syndrome, conspicuous and pathological findings possibly indicating the presence of underlying Marfan syndrome were assessed. Several systemic features were scored that supported the presence of underlying Marfan syndrome in this mummy. These findings were: pectus carinatum and chest asymmetry, dural ectasia, protrusio acetabuli, dolichocephaly, down-slanting palpebral fissures, malar hypoplasia and (probable) reduced elbow extension. Aortic dissection, a cardinal feature of Marfan syndrome, turned out to be the diagnostic key for the paleoradiological diagnosis of this disease. The demonstrated CT findings contribute to the spectrum of cardiovascular diseases and inherited connective tissue disease in the fields of paleopathology and paleoradiology. Copyright © 2018 Elsevier Inc. All rights reserved.

Circulatory CNP Rescues Craniofacial Hypoplasia in Achondroplasia.

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Yamanaka, S; Nakao, Kazumasa; Koyama, N; Isobe, Y; Ueda, Y; Kanai, Y; Kondo, E; Fujii, T; Miura, M; Yasoda, A; Nakao, Kazuwa; Bessho, K

2017-12-01

Achondroplasia is the most common genetic form of human dwarfism, characterized by midfacial hypoplasia resulting in occlusal abnormality and foramen magnum stenosis, leading to serious neurologic complications and hydrocephalus. Currently, surgery is the only way to manage jaw deformity, neurologic complications, and hydrocephalus in patients with achondroplasia. We previously showed that C-type natriuretic peptide (CNP) is a potent stimulator of endochondral bone growth of long bones and vertebrae and is also a potent stimulator in the craniofacial region, which is crucial for midfacial skeletogenesis. In this study, we analyzed craniofacial morphology in a mouse model of achondroplasia, in which fibroblast growth factor receptor 3 (FGFR3) is specifically activated in cartilage ( Fgfr3 ach mice), and investigated the mechanisms of jaw deformities caused by this mutation. Furthermore, we analyzed the effect of CNP on the maxillofacial area in these animals. Fgfr3 ach mice exhibited midfacial hypoplasia, especially in the sagittal direction, caused by impaired endochondral ossification in craniofacial cartilage and by premature closure of the spheno-occipital synchondrosis, an important growth center in craniomaxillofacial skeletogenesis. We crossed Fgfr3 ach mice with transgenic mice in which CNP is expressed in the liver under the control of the human serum amyloid-P component promoter, resulting in elevated levels of circulatory CNP ( Fgfr3 ach /SAP-Nppc-Tg mice). In the progeny, midfacial hypoplasia in the sagittal direction observed in Fgfr3 ach mice was improved significantly by restoring the thickness of synchondrosis and promoting proliferation of chondrocytes in the craniofacial cartilage. In addition, the foramen magnum stenosis observed in Fgfr3 ach mice was significantly ameliorated in Fgfr3 ach /SAP-Nppc-Tg mice due to enhanced endochondral bone growth of the anterior intraoccipital synchondrosis. These results clearly demonstrate the therapeutic

Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism

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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

Method for imaging pulmonary arterial hypoplasia

International Nuclear Information System (INIS)

Triantafillou, M.

2000-01-01

Full text: Pulmonary hypoplasia represents an incomplete development of the lung, resulting in the reduction of distended lung volume. This is associated with small or absent number of airway divisions, alveoli, arteries and veins. Unilateral pulmonary Hypoplasia is often asymptomatic and may be demonstrated as a hypodense lung on a chest X-ray. Computer Tomography (CT) scanning would show anatomical detail and proximal vessels. Magnetic Resonance Imaging (MRI) will show no more detail than which the CT scan has already demonstrated. It is, also, difficult to visualise collateral vessels from systemic and/or bronchial vessels on both these modalities. Pulmonary Angiography would give the definitive answer, but it is time consuming and has significant risks associated with the procedure. There are high costs associated with these modalities. Nuclear Medicine Ventilation/Perfusion (V/Q) scan performed on these patients would demonstrate diminished ventilation due to reduced lung volume and absence of perfusion to the hypoplastic lung. To date, we have performed V/Q lung scan on two children in our department. Both cases demonstrate diminished ventilation with no perfusion to the hypoplastic lung. Though the gold standard is Pulmonary Angiography, V/Q scanning is cost effective, less time consuming and a non invasive procedure that can be performed as an outpatient. It is accurate as it demonstrates absent lung perfusion, confirming the patient has pulmonary arterial hypoplasia. Copyright (2000) The Australian and New Zealand Society of Nuclear Medicine Inc

Regional disc change in segmental hypoplasia of the lumbosacral vertebral bodies: MR findings

International Nuclear Information System (INIS)

Kim, Sung Kyu; Lee Seung Ro; Moon, Won Jin; Park, Dong Woo; Hahm, Chang Kok

2000-01-01

To classify types of vertebral hypoplasia and to investigate the prevalence and patterns of associated disc degeneration. Defining vertebral hypoplasia as occurring when the AP diameter of a lower vertebral body is smaller than that of an upper ones, we retrospectively reviewed the MR images obtained in 34 cases of this condition involving young adults. Two major types and two subtypes, a total of four different entities were classified as follows; type I: hypoplasia involving a single vertebral body; type II: hypoplasia involving serial lower segmental vertebral bodies; subtype a: hypoplastic body located anteriorly along the anterior spinal line; subtype b: hypoplastic body located posteriorly along the posterior spinal line. We also investigated each type of vertebral hypoplasia and patterns of associated disc changes. Three different types were observed. In type IIa (n=3D29), posterior disc occurred in 8/29 cases, diffuse degeneration in 21/29 patients, and posterior disc herniation in all. All type Ia cases (3/3) showed diffuse disc degeneration at both upper and lower disc levels, with posterior disc herniation, while both type IIb cases (2/2) showed diffuse disc degeneration, with bidirectional disc herniation. By identifying the exact patterns of vertebral hypoplasia, we were able to predict which portion of the disc was likely to degenerate. (author)

Diagnosis, imaging and clinical management of aortic coarctation.

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Dijkema, Elles J; Leiner, Tim; Grotenhuis, Heynric B

2017-08-01

Coarctation of the aorta (CoA ) is a well-known congenital heart disease (CHD) , which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of C o A remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (> 20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV). Transthoracic echocardiography is the primary imaging modality for suspected CoA. However, cardiac magnetic resonance imaging is the preferred advanced imaging modality for non-invasive diagnosis and follow-up of CoA. Adequate and timely diagnosis of CoA is crucial for good prognosis, as early treatment is associated with lower risks of long-term morbidity and mortality. Numerous surgical and transcatheter treatment strategies have been reported for CoA. Surgical resection is the treatment of choice in neonates, infants and young children. In older children (> 25 kg) and adults, transcatheter treatment is the treatment of choice. In the current era, patients with CoA continue to have a reduced life expectancy and an increased risk of cardiovascular sequelae later in life, despite adequate relief of the aortic stenosis. Intensive and adequate follow-up of the left ventricular function, valvular function, blood pressure and the anatomy of the heart and the aorta are , therefore, critical in the management of CoA. This review provides an overview of the current state-of-the-art clinical diagnosis, diagnostic imaging algori thms, treatment and follow-up of patients with CoA. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Unilateral hypoplasia with contralateral hypertrophy of anterior belly of digastric muscle: a case report.

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Ochoa-Escudero, Martin; Juliano, Amy F

2016-10-01

Anomalies of the anterior belly of the digastric muscle (DM) are uncommon. We present a case of hypoplasia of the anterior belly of the left DM with hypertrophy of the anterior belly of the contralateral DM. The importance of recognizing this finding is to differentiate hypoplasia of the anterior belly of the DM from denervation atrophy, and not to confuse contralateral hypertrophy with a submental mass or lymphadenopathy. In denervation atrophy of the anterior belly of the DM, associated atrophy of the ipsilateral mylohyoid muscle is present. Hypertrophy of the anterior belly of the contralateral DM can be differentiated from a submental mass or lymphadenopathy by recognizing its isodensity on computed tomography and isointensity on magnetic resonance imaging to other muscles, without abnormal contrast enhancement.

Goldenhar Syndrome with Dextrocardia and Right Pulmonary Hypoplasia: An Unusual Association

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Nagendra Chaudhary

2017-01-01

Full Text Available Goldenhar syndrome (GS, a rare condition, occurring due to defect in development of first and second branchial arches, is characterized by a combination of various anomalies involving face, eyes, ears, vertebrae, heart, and lungs. The etiology of GS is not fully known, although various hypotheses have been proposed along with its genetic association and many other causes. Facial asymmetry and hypoplasia of the mandible are characteristic features of GS along with microtia and preauricular appendages and pits. Dextrocardia or pulmonary hypoplasia in GS has previously been reported separately. We report a 7-year-old female child of GS with combination of anomalies, dextrocardia, and pulmonary hypoplasia, which is a rare association.

Genetics Home Reference: Leydig cell hypoplasia

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... normal male sexual development before birth and during puberty. In Leydig cell hypoplasia , affected individuals with a typical male chromosomal pattern (46,XY) may have a range of genital abnormalities. Affected males may have a small penis (micropenis), the opening of the urethra on the ...

Glenoid hypoplasia: a report of 2 patients.

Science.gov (United States)

Lynch, Christopher J; Taylor, John A M; Buchberger, Dale J

2008-06-01

This article discusses the imaging findings, clinical findings, and conservative chiropractic management of 2 patients with glenoid hypoplasia. Conventional radiographs of both patients revealed a hypoplastic glenoid bilaterally. Notch-like defects along with signs of degenerative disease were evident within the lower portion of the glenoid rims bilaterally in 1 patient and in the left glenoid rim of the other patient. Magnetic resonance imaging revealed a degenerative cyst or cortical defect in one patient along the anterior humeral head. The second patient showed a small slightly lobulated cystic region just posterior to the glenoid rim, consistent with the appearance of a synovial or ganglion cyst. Computed tomography with 3-dimensional reconstruction in 1 patient confirmed the presence of large posterior and superior osteophytes arising from the significantly hypoplastic glenoid. These images also revealed a slight posterior subluxation of the humeral head, widening of the anterior glenohumeral joint space, and retroversion of the glenoid. Treatment consisted of manual joint manipulation, soft tissue therapies, and therapeutic exercise for both patients. Both patients experienced improvements in symptoms, function, and physical examination findings. Glenoid hypoplasia is a developmental anomaly of the scapular neck which is predominantly bilateral and symmetric. Cross-sectional imaging studies should be considered in patients with symptoms that fail to improve over time. Conservative chiropractic care may be effective in managing symptoms in patients with glenoid hypoplasia.

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

International Nuclear Information System (INIS)

Manchev, I.; Manolova, T.; Manchev, L.

2015-01-01

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

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

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Kamran, Mudassar, E-mail: kamranm@mir.wustl.edu; Fowler, Kathryn J., E-mail: fowlerk@mir.wustl.edu; Mellnick, Vincent M., E-mail: mellnickv@mir.wustl.edu [Washington University School of Medicine, Mallinckrodt Institute of Radiology (United States); Sicard, Gregorio A., E-mail: sicard@wudosis.wustl.edu [Washington University School of Medicine, Department of Surgery (United States); Narra, Vamsi R., E-mail: narrav@mir.wustl.edu [Washington University School of Medicine, Mallinckrodt Institute of Radiology (United States)

2016-06-15

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.

Left Ventricular Assist Device Implantation with Concomitant Aortic Valve and Ascending Aortic Replacement.

Science.gov (United States)

Huenges, Katharina; Panholzer, Bernd; Cremer, Jochen; Haneya, Assad

2018-01-01

Left ventricular assist device (LVAD) is nowadays a routine therapy for patients with advanced heart failure. We present the case of a 74-year-old male patient who was admitted to our center with terminal heart failure in dilated cardiomyopathy and ascending aortic aneurysm with aortic valve regurgitation. The LVAD implantation with simultaneous aortic valve and supracoronary ascending aortic replacement was successfully performed.

Management of aortic graft infections - the present strategy and future perspectives.

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Treska, V; Certik, B; Molacek, J

2016-01-01

Aortic graft infections (AGI) are serious complications of open and endovascular types of surgery with an incidence rate of 0.6-3 %. AGI are associated with 30-60 % perioperative mortality and 40-60 % morbidity rate with limb amputation rates between 10 % and 40 %. The economic cost of AGI is substantial. At the time of aortic reconstruction, almost 90 % of patients have one or more predisposing factors for AGI. The diagnosis is based on clinical symptomatology, laboratory markers, microbial cultures, and imaging modalities. The general principle of surgical treatment lies in the removal of infected graft, debridement of infected periprosthetic tissues, and vascular reconstruction by in situ or extra-anatomic bypass with long-term antibiotic therapy. The conservative treatment is used only for selected patients with endograft infection. This review summarizes the current knowledge about the incidence, predisposing factors, etiology, diagnosis, treatment options, and prevention of aortic vascular graft and endograft infections. With the growing number of endovascular procedures we can expect more cases of infected aortic endografts in patients with severe comorbidities in the near future, where the recent radical surgical approach (graft excision, debridement, and new revascularization) cannot be used. Therefore the less invasive, sophisticated and individualized treatment strategies will have to be used in search of the best therapeutic approach to each specific patient (Fig. 4, Ref. 82).

Pontocerebellar hypoplasia associated with respiratory-chain defects

NARCIS (Netherlands)

de Koning, T. J.; de Vries, L. S.; Groenendaal, F.; Ruitenbeek, W.; Jansen, G. H.; Poll-The, B. T.; Barth, P. G.

1999-01-01

Pontocerebellar hypoplasias are congenital disorders of brain morphogenesis which include such diverse etiologies as carbohydrate-deficient glycoprotein syndrome type 1, cerebromuscular dystrophies (Walker-Warburg syndrome, Fukuyama syndrome, muscle-eye-brain disease) and at least two types of

Ectopic KIT copy number variation underlies impaired migration of primordial germ cells associated with gonadal hypoplasia in cattle (Bos taurus.

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Heli Venhoranta

Full Text Available Impaired migration of primordial germ cells during embryonic development causes hereditary gonadal hypoplasia in both sexes of Northern Finncattle and Swedish Mountain cattle. The affected gonads exhibit a lack of or, in rare cases, a reduced number of germ cells. Most affected animals present left-sided gonadal hypoplasia. However, right-sided and bilateral cases are also found. This type of gonadal hypoplasia prevails in animals with white coat colour. Previous studies indicated that gonadal hypoplasia is inherited in an autosomal recessive fashion with incomplete penetrance. In order to identify genetic regions underlying gonadal hypoplasia, a genome-wide association study (GWAS and a copy number variation (CNV analysis were performed with 94 animals, including 21 affected animals, using bovine 777,962 SNP arrays. The GWAS and CNV results revealed two significantly associated regions on bovine chromosomes (BTA 29 and 6, respectively (P=2.19 x 10(-13 and P=5.65 x 10(-6. Subsequent cytogenetic and PCR analyses demonstrated that homozygosity of a ~500 kb chromosomal segment translocated from BTA6 to BTA29 (Cs29 allele is the underlying genetic mechanism responsible for gonadal hypoplasia. The duplicated segment includes the KIT gene that is known to regulate the migration of germ cells and precursors of melanocytes. This duplication is also one of the two translocations associated with colour sidedness in various cattle breeds.

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

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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.

Trans-sinusal maxillary distraction for correction of midfacial hypoplasia: long-term clinical results.

NARCIS (Netherlands)

Nadjmi, N.; Schutyser, F.A.C.; Erum, R. van

2006-01-01

Maxillary distraction osteogenesis is indicated in severe angle class III malocclusions, and severe maxillary hypoplasia among some cleft patients and other craniofacial deformities. Twenty patients, aged 8-48 years (mean 17.8+/-10.5 SD) with maxillary and midfacial hypoplasia were treated. The

Correction of axial deformity during lengthening in fibular hypoplasia: Hexapodal versus monorail external fixation.

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Chalopin, A; Geffroy, L; Pesenti, S; Hamel, A; Launay, F

2017-09-01

Childhood fibular hypoplasia is a rare pathology which may or may not involve limb-length discrepancy and axial deformity in one or more dimensions. The objective of the present study was to compare the quality of the axial correction achieved in lengthening procedures by hexapodal versus monorail external fixators. The hypothesis was that the hexapodal fixator provides more precise correction. A retrospective multicenter study included 52 children with fibular hypoplasia. Seventy-two tibias were analyzed, in 2 groups: 52 using a hexapodal fixator, and 20 using a monorail fixator. Mean age was 10.2 years. Mean lengthening was 5.7cm. Deformities were analyzed and measured in 3 dimensions and classified in 4 preoperative types and 4 post-lengthening types according to residual deformity. Complete correction was achieved in 26 tibias in the hexapodal group (50%) and 2 tibias in the monorail group (10%). Mean post-correction mechanical axis deviation was smaller in the hexapodal group: 12.83mm, versus 14.29mm in the monorail group. Mean post-correction mechanical lateral distal femoral angle was 87.5° in the hexapodal group, versus 84.3° in the monorail group (P=0.002), and mean mechanical medial proximal tibial angle 86.9° versus 89.5°, respectively (P=0.015). No previous studies focused on this congenital pathology in lengthening and axial correction programs for childhood lower-limb deformity. The present study found the hexapodal fixator to be more effective in conserving or restoring mechanical axes during progressive bone lengthening for fibular hypoplasia. The hexapodal fixator met the requirements of limb-length equalization in childhood congenital lower-limb hypoplasia, providing better axial correction than the monorail fixator. IV. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Pulmonary Hypoplasia Caused by Fetal Ascites in Congenital Cytomegalovirus Infection Despite Fetal Therapy

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Kazumichi Fujioka

2017-11-01

Full Text Available We report two cases of pulmonary hypoplasia due to fetal ascites in symptomatic congenital cytomegalovirus (CMV infections despite fetal therapy. The patients died soon after birth. The pathogenesis of pulmonary hypoplasia in our cases might be thoracic compression due to massive fetal ascites as a result of liver insufficiency. Despite aggressive fetal treatment, including multiple immunoglobulin administration, which was supposed to diminish the pathogenic effects of CMV either by neutralization or immunomodulatory effects, the fetal ascites was uncontrollable. To prevent development of pulmonary hypoplasia in symptomatic congenital CMV infections, further fetal intervention to reduce ascites should be considered.

Genetics Home Reference: VLDLR-associated cerebellar hypoplasia

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... hypoplasia include moderate to profound intellectual disability, impaired speech (dysarthria) or a lack of speech, and eyes that do not look in the ... the United States. This condition has also been reported in families from Iran and Turkey. Related Information ...

Osteopathia striata: a characteristic X-ray finding in focal dermal hypoplasia (Goltz-Gorlin syndrome)

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Barthels, W.; Boepple, D.; Petzel, H.

1982-12-01

Two cases of the very rare Goltz-Gorlin syndrome are presented. The relationship of osteopathia striata and focal dermal hypoplasia is discussed, and it is concluded that the osteopathia striata represents the characteristic picture of this ectopic mesodermal abnormality.

Diffuse large B-cell lymphoma chemotherapy reveals a combined immunodeficiency syndrome in cartilage hair hypoplasia.

Science.gov (United States)

Nguyen, Alexandre; Martin Silva, Nicolas; de Boysson, Hubert; Damaj, Gandhi; Aouba, Achille

2018-04-24

Cartilage hair hypoplasia (CHH) is a rare autosomal recessive ribosomopathy characterised by skeletal and integumentary system manifestations. It may also present with varied forms and intensities of haematopoietic and/or immune disorders. We report a 27-year-old female who presented a picture of combined immunodeficiency after receiving an adriamycin-based chemotherapy regimen followed by autologous stem cell transplantation. Her medical history indicated neonatal dwarfism, recurrent ear, nose and throat and respiratory infections, and hypogammaglobulinaemia, which were suggestive of a primary minor B-cell immune deficiency. Taken together, the diagnosis of cartilage hair hypoplasia was suspected and confirmed by means of molecular biological analysis. Here, we discuss the causal relationship and molecular mechanisms existing between both primary immunodeficiency and lymphoma conditions and between chemotherapy cytotoxicity and aggravation of the immune system and associated hematopoietic dysfunction, considering the role of all these components in light of the initially undiagnosed cartilage hair hypoplasia. Finally, this case highlights the importance of screening for primary immunodeficiencies in the setting of a diagnosis of lymphoma and/or dwarfism; moreover, CHH must be distinguished from other causes of small size; its diagnosis and complete check-up must include the molecular characterisation, and its management must be global in collaboration with haematologists, immunologists and internists.

Osteopathia striata: A characteristic X-ray finding in focal dermal hypoplasia (Goltz-Gorlin syndrome)

International Nuclear Information System (INIS)

Barthels, W.; Boepple, D.; Petzel, H.

1982-01-01

Two cases of the very rare Goltz-Gorlin syndrome are presented. The relationship of osteopathia striata and focal dermal hypoplasia is discussed, and it is concluded that the osteopathia striata represents the characteristic picture of this ectopic mesodermal abnormality. (orig.)

Loyes-Dietz syndrome presenting with severe aortic insufficiency – case report

International Nuclear Information System (INIS)

Kunovsky, P.; Dinka, R.; Krissakova, A.; Culen, M.; Nosal, M.; Kovacik, J.; Ilencikova, D.; Outrata, R.

2013-01-01

Severe aortic insufficiency (AI) in childhood is very rare cause of heart failure. Mostly is associated with connective tissue disorders as Marfan syndrome (MFS) or recently described Loyes-Dietz syndrome (LDS) (1). Authors present a case report of 9 years old girl with severe AI caused by aneurysm of aortic root. Typical findings associated with LDS are wide spread aneurysms of aorta, hypertelorism, cleft palate or split uvula (bifida uvula) and generalized arterial tortuosity. LDS is an autosomal dominant genetic disorder of the connective tissue; caused by mutation in the genes encoding the transforming growth factor beta receptor 1 and 2 (TGFBR1 and TGFRB2). Afflicted patients demonstrate different involvement of cardiovascular, musculoskeletal and central nervous system. From prognostic point of view the most consequential is widespread involvement of arterial system, when in addition to ascending aorta other parts of aorta and their branches might be also afflicted. Life threatening dissection and ruptures can occur earlier and at less dilated aneurysms than in MFS, requiring more aggressive diagnostic and therapeutic management with timely surgical intervention. Incidence of LDS is less frequent than serious congenital heart defects but due to its catastrophic potential even in early childhood as well as possible preventive intervention the importance of early diagnosis and treatment should be emphasized. (author)

Traumatic Aortic Injury

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Brianna Miner

2016-09-01

Full Text Available History of present illness: A 48-year-old male with unknown past medical history presents as a trauma after being hit by a car traveling approximately 25 miles per hour. On initial presentation, the patient is confused, combative, and not answering questions appropriately. The patient is hypotensive with a blood pressure of 68/40 and a heart rate of 50 beats per minute, with oxygen saturation at 96% on room air. FAST scan is positive for fluid in Morrison’s pouch, splenorenal space, and pericardial space. Significant findings: The initial chest x-ray showed an abnormal superior mediastinal contour (blue line, suggestive of a possible aortic injury. The CT angiogram showed extensive circumferential irregularity and outpouching of the distal aortic arch (red arrows compatible with aortic transection. In addition, there was a circumferential intramural hematoma, which extended through the descending aorta to the proximal infrarenal abdominal aorta (green arrow. There was also an extensive surrounding mediastinal hematoma extending around the descending aorta and supraaortic branches (purple arrows. Discussion: Traumatic aortic injury is a life-threatening event. The incidence of blunt thoracic aortic injury is low, between 1 to 2 percent of those patients with blunt thoracic trauma.1 However, approximately 80% of patients with traumatic aortic injury die at the scene.2 Therefore it is imperative to diagnose traumatic aortic injury in a timely fashion. The diagnosis can be difficult due to the non-specific signs and symptoms and other distracting injuries. Clinical suspicion should be based on the mechanism of the injury and the hemodynamic status of the patient. In any patient with blunt or penetrating trauma to the chest that is hemodynamically unstable, traumatic aortic injury should be on the differential. Chest x-ray can be used as a screening tool. A normal chest x-ray has a negative predictive value of approximately 97%. CTA chest is the

WDR73 missense mutation causes infantile onset intellectual disability and cerebellar hypoplasia in a consanguineous family.

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Jiang, Chen; Gai, Nan; Zou, Yongyi; Zheng, Yu; Ma, Ruiyu; Wei, Xianda; Liang, Desheng; Wu, Lingqian

2017-01-01

Galloway-Mowat syndrome (GMS) is a very rare autosomal-recessive disorder characterized by nephrotic syndrome associated with microcephaly, and various central nervous system abnormalities, mostly cerebral hypoplasia or cerebellar atrophy, intellectual disability and neural-migration defects. WDR73 is the only gene known to cause GMS, and has never been implicated in other disease. Here we present a Chinese consanguineous family with infantile onset intellectual disability and cerebellar hypoplasia but no microcephaly. Whole exome sequencing identified a WDR73 p.W371G missense mutation. The mutation is confirmed to be segregated in this family by Sanger sequencing according to a recessive inheritance pattern. It is predicted to be deleterious by multiple algorithms and affect highly conserved site. Structural modeling revealed conformational differences between the wild type protein and the p.W371G protein. Real-time PCR and Western blotting revealed altered mRNA and protein levels in mutated samples. Our study indicates the novel WDR73 p.W371G missense mutation causes infantile onset intellectual disability and cerebellar hypoplasia in recessive mode of inheritance. Our findings imply that microcephaly is a variable phenotype in WDR73-related disease, suggest WDR73 to be a candidate gene of severe intellectual disability and cerebellar hypoplasia, and expand the molecular spectrum of WDR73-related disease. Copyright © 2016 Elsevier B.V. All rights reserved.

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

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Masuda, Naonori; Hasegawa, Taiji; Yamashita, Mariko; Ogata, Nahoko

2014-01-01

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.

Management of Cleft Maxillary Hypoplasia with Anterior Maxillary Distraction: Our Experience

OpenAIRE

Chacko, Tojan; Vinod, Sankar; Mani, Varghese; George, Arun; Sivaprasad, K. K.

2013-01-01

Maxillary hypoplasia is a common developmental problem in cleft lip and palate deformities. Since 1970s these deformities have traditionally been corrected by means of orthognathic surgery. Management of skeletal deformities in the maxillofacial region has been an important challenge for maxillofacial surgeons and orthodontists. Distraction osteogenesis is a surgical technique that uses body’s own repairing mechanisms for optimal reconstruction of the tissues. We present four cases of anterio...

Analysis of the enamel hypoplasia using micro-CT scanner versus classical method.

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Marchewka, Justyna; Skrzat, Janusz; Wróbel, Andrzej

2014-01-01

This article demonstrates the use of micro-CT scanning of the teeth surface for recognizing and evaluating severity of the enamel hypoplasia. To test capabilities of the microtomography versus classical method of evaluation hypoplastic defects of the enamel we selected two human teeth (C, M(2)) showing different types of enamel hypoplasia: linear, pits, and groove. Examined samples derive from archeological material dated on XVII-XVIII AD and excavated in Poland. In the current study we proved that micro-CT scanning is a powerful technique not only for imaging all kinds of the enamel hypoplasia but also allows to perform accurate measurements of the enamel defects. We figure out that contrary to the classical method of scoring enamel defects, the micro-computed tomography yields adequate data which serve for estimating the length of stress episode and length of interval between them.

Urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome: long-term results.

Science.gov (United States)

Kajbafzadeh, Abdol-Mohammad; Rasouli, Mohammad Reza; Dianat, SeyedSaeid; Nezami, Behtash G; Mahboubi, Amir Hassan; Sina, Alireza

2010-11-01

The aim of the study was to evaluate the efficacy and safety of urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome (PBS). During a 10-year period, 7 infants with PBS and urethral hypoplasia presented either with open urachus or surgically created urinary diversion referred to our hospital. Five milliliters of normal saline was pushed via a 22-gauge plastic angiocatheter into the urethra with simultaneous finger pressure on the perineum to occlude the proximal urethra that was repeated with higher volumes of the solution (up to 20 mL). The procedure was continued until a 6F or 8F feeding tube catheter confirmed the urethral patency. Hydrodistension was repeated in 3-month intervals till complete patency was confirmed by imaging. Median age of the infants was 6 (1-8) months. All urethral hydrodistension were successful after 1 to 3 sessions. Follow-up imaging studies showed significant improvement in all patients except one. Natural and surgically created urinary diversions were closed in 6 infants. The hydrodistension create an equal and constant pressure into the urethral wall without any urethral damage. This technique can be considered along with the other available methods for management of urethral hypoplasia in selected cases of PBS. Copyright © 2010 Elsevier Inc. All rights reserved.

Rhabdomyolysis in pontocerebellar hypoplasia type 2 (PCH-2)

NARCIS (Netherlands)

Barth, Peter G.; Ryan, Monique M.; Webster, Richard I.; Aronica, Eleonora; Kan, Alex; Ramkema, Marja; Jardine, Philip; Poll-The, Bwee Tien

2008-01-01

Pontocerebellar hypoplasia type 2, an autosomal recessive neurodegeneration with prenatal onset, is characterised by progressive microcephaly and chorea/dystonia and has not previously been associated with muscular involvement. The gene associated with PCH-2 is unknown. An episode of rhabdomyolysis

Bone marrow hypoplasia associated with fenbendazole administration in a dog.

Science.gov (United States)

Gary, Anthony T; Kerl, Marie E; Wiedmeyer, Charles E; Turnquist, Susan E; Cohn, Leah A

2004-01-01

A 1.5-year-old Doberman pinscher was presented with sudden-onset of fever and malaise. Twelve days prior to presentation, fenbendazole therapy was initiated for a suspected lungworm infection. Results of a complete blood count on presentation showed pancytopenia, while histopathological evaluation of a bone marrow core sample revealed bone marrow hypoplasia of undetermined etiology. Bactericidal antibiotics and fluid therapy, as well as discontinuation of fenbendazole administration, led to a complete resolution of clinical and hematological abnormalities within 15 days. An idiosyncratic reaction to fenbendazole was suspected based on the absence of infectious, neoplastic, autoimmune, and toxic etiologies, as well as resolution of clinical signs and pancytopenia upon drug withdrawal.

Acute Type A Aortic Dissection Successfully Managed with One-stage Surgery of Total Aortic Arch Replacement with Supra-aortic Transposition Plus Frozen Elephant Trunk Technique

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Meng-Lin Lee

2014-09-01

Full Text Available Acute type A aortic dissection has long been a challenging issue. The surgical techniques traditionally vary with the anatomic extent of the aortic dissection. Simple ascending aortic grafting can be lifesaving, but the lesions beyond the aorta, which include the arch vessels and descending aorta, remain potential hazards. In this paper, we present a patient in which acute type A aortic dissection with lesions extending into descending thoracic aorta was successfully managed by total arch replacement with supra-aortic transposition plus the frozen elephant trunk technique to the descending aorta. A 67-year-old gentleman presented with severe tearing pain from the anterior to posterior chest. Computed tomography confirmed the diagnosis of acute type A dissection extending to the level of the right common iliac artery. An emergent operation was performed as in the aforementioned technique. The surgery went well and the patient was discharged without comorbidities on postoperative day 25. The patient had regular outpatient clinical follow-up. The follow-up computed tomography images showed adequate results with the obliteration of the false lumen. In conclusion, total aortic arch replacement with supra-aortic transposition plus frozen elephant trunk technique is a safe and feasible operative method for patients with detrimental acute type A aortic dissection.

Intertooth patterns of hypoplasia expression: implications for childhood health in the classic Maya collapse.

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Wright, L E

1997-02-01

Enamel hypoplasias, which record interacting stresses of nutrition and illness during the period of tooth formation, are a key tool in the study of childhood health in prehistory. But interpretation of the age of peak morbidity is complicated by differences in susceptibility to stress both between tooth positions and within a single tooth. Here, hypoplasias are used to evaluate the prevailing ecological model for the collapse of Classic Period Lowland Maya civilization, circa AD 900. Hypoplasias were recorded in the full dentition of 160 adult skeletons from six archaeological sites in the Pasion River region of Guatemala. Instead of constructing a composite scale of stress experience, teeth are considered separately by position in the analysis. No statistical differences are found in the proportion of teeth affected by hypoplasia between "Early," Late Classic, and Terminal Classic Periods for anterior teeth considered to be most susceptible to stress, indicating stability in the overall stress loads affecting children of the three chronological periods. However, hypoplasia trends in posterior teeth may imply a change in the ontogenetic-timing of more severe stress episodes during the final occupation and perhaps herald a shift in child-care practices. These results provide little support for the ecological model of collapse but do call to attention the potential of posterior teeth to reveal subtle changes in childhood morbidity when consideredindividually.

Hybrid treatment of penetrating aortic ulcer

International Nuclear Information System (INIS)

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

2015-01-01

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

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)

Bovine aortic arch: A novel association with thoracic aortic dilation

International Nuclear Information System (INIS)

Malone, C.D.; Urbania, T.H.; Crook, S.E.S.; Hope, M.D.

2012-01-01

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.

Causes of congenital unilateral pulmonary hypoplasia

International Nuclear Information System (INIS)

Currarino, G.; Williams, B.; Children's Medical Center, Dallas, TX

1985-01-01

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.)

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

Science.gov (United States)

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

2017-09-01

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

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

OpenAIRE

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

2003-01-01

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

X-linked adrenal hypoplasia congenita: a case report and ethical dilemma.

Science.gov (United States)

Ismail, Heba M; Rincon, Marielisa

2014-07-01

Our objective is to present the first case report of X-linked adrenal hypoplasia congenita in a child conceived by a donated egg and which also presented atypically, with initial mineralocorticoid deficiency. Case report with literature review. A late preterm fraternal twin male, conceived by in vitro fertilization of donated eggs, presented shortly after birth with feeding intolerance, hyponatremia, and hyperkalemia. Testing revealed a low aldosterone level, high plasma renin activity, normal cortisol level, and normal 17-hydroxyprogesterone level. He was diagnosed with 18-hydroxylase deficiency based on low 18-hydroxycorticosterone levels and was treated with mineralocorticoid successfully for 17 months. At age 18 months, he presented with dehydration secondary to herpetic gingivostomatitis and was found to be hypoglycemic, hyponatremic, hyperkalemic, and acidotic, with a low serum cortisol level. An adrenocorticotropic hormone (ACTH) stimulation test revealed low levels of all adrenal cortex products, with an elevated ACTH level. He was started on glucocorticoids. Genetic testing confirmed X-linked adrenal hypoplasia congenita (AHC). His asymptomatic fraternal twin underwent genetic testing and the results were negative. The fertility center records indicated that the mother had donated eggs to other families, but none of the children were known to have this disorder. The egg donor was informed but did not pursue genetic testing. We report a case of X-linked AHC presenting in the context of extraordinary ethical considerations. Our case raises a question unique to the era of assisted reproduction: should routine genetic screening of gamete donors be done for rare but potentially life-threatening conditions?

Patients With Type A Acute Aortic Dissection Presenting With an Abnormal Electrocardiogram.

Science.gov (United States)

Costin, Nathaniel I; Korach, Amit; Loor, Gabriel; Peterson, Mark D; Desai, Nimesh D; Trimarchi, Santi; de Vincentiis, Carlo; Ota, Takeyoshi; Reece, T Brett; Sundt, Thoralf M; Patel, Himanshu J; Chen, Edward P; Montgomery, Dan G; Nienaber, Christoph A; Isselbacher, Eric M; Eagle, Kim A; Gleason, Thomas G

2018-01-01

The electrocardiogram (ECG) is often used in the diagnosis of patients presenting with chest pain to emergency departments. Because chest pain is a common manifestation of type A acute aortic dissection (TAAAD), ECGs are obtained in much of this population. We evaluated the effect of particular ECG patterns on the diagnosis and treatment of TAAAD. TAAAD patients (N = 2,765) enrolled in the International Registry of Acute Aortic Dissection were stratified based on normal (n = 1,094 [39.6%]) and abnormal (n = 1,671 [60.4%]) findings on presenting ECGs and further subdivided according to specific ECG findings. Time data are presented in hours as medians (quartile 1 to quartile 3). Patients with ECGs with abnormal findings presented to the hospital sooner after symptom onset than those with ECGs with normal findings (1.4 [0.8 to 3.3] vs 2.0 [1.0 to 3.3]; p = 0.005). Specifically, this was seen in patients with infarction with new Q waves or ST elevation (1.3 [0.6 to 2.7] vs 1.5 [0.8 to 3.3]; p = 0.049). Interestingly, the time between symptom onset and diagnosis was longer with infarction with old Q waves (6.7 [3.2 to 18.4] vs 5.0 [2.9 to 11.8]; p = 0.034) and nonspecific ST-T changes (5.8 [3.0 to 13.8] vs 4.5 [2.8 to 10.5]; p = 0.002). Surgical mortality was higher in patients with abnormal ECG findings (20.6% vs 11.9%, p presenting with abnormal ECG results are sicker, have more in-hospital complications, and are more likely to die. The frequency of nonspecific ST-T abnormalities and its association with delay in diagnosis and treatment presents an opportunity for practice improvement. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Bilateral familial vertical Duane Syndrome with synergistic convergence, aberrant trigeminal innervation, and facial hypoplasia

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Malvika Gupta

2014-01-01

Full Text Available A 5-year-old girl presented with bilateral familial vertical  Duane retraction syndrome with alternating esotropia, elevation deficit, Marcus gunn phenomenon, and facial hypoplasia. Abnormal adducting downshoots on attempting abduction suggestive of a synergistic convergence were noted. Hypothesis suggests aberrant innervations or peripheral anatomic connections between inferior and medial recti.

Optic nerve hypoplasia

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Savleen Kaur

2013-01-01

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

Application of thoracic endovascular aortic repair (TEVAR) in treating dwarfism with Stanford B aortic dissection

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Qiu, Jian; Cai, Wenwu; Shu, Chang; Li, Ming; Xiong, Qinggen; Li, Quanming; Li, Xin

2018-01-01

Abstract Rationale: To apply thoracic endovascular aortic repair (TEVAR) to treat dwarfism complicated with Stanford B aortic dissection. Patient concerns: In this report, we presented a 63-year-old male patient of dwarfism complicated with Stanford B aortic dissection successfully treated with TEVAR. Diagnoses: He was diagnosed with dwarfism complicated with Stanford B aortic dissection. Interventions: After conservative treatment, the male patient underwent TEVAR at 1 week after hospitalization. After operation, he presented with numbness and weakness of his bilateral lower extremities, and these symptoms were significantly mitigated after effective treatment. At 1- and 3-week after TEVAR, the aorta status was maintained stable and restored. Outcomes: The patient obtained favorable clinical prognosis and was smoothly discharged. During subsequent follow-up, he remained physically stable. Lessons: TEVAR is probably an option for treating dwarfism complicated with Stanford B aortic dissection, which remains to be validated by subsequent studies with larger sample size. PMID:29703033

Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

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Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden [Istanbul Faculty of Medicine, Department of Radiology, Istanbul (Turkey); Ozmen, Meral [Istanbul Faculty of Medicine, Department of Paediatric Neurology, Istanbul (Turkey)

2004-11-01

Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)

Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

International Nuclear Information System (INIS)

Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden; Ozmen, Meral

2004-01-01

Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)

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

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

2011-05-15

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

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

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Stefan Gysin

2015-05-01

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

Application of mitomycin C after endoscopic lysis of congenital laryngeal web combined with epiglottic hypoplasia in a middle-aged man.

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Roh, Jong-Lyel

2006-04-01

Laryngeal webs and epiglottic hypoplasias are uncommon congenital anomalies. Anterior glottic web combined with epiglottic hypoplasia was found in a middle-aged man presenting with hoarseness and dyspnea on exertion. This can be considered as a unique isolated defect of the larynx during early fetal development. The laryngeal web can be successfully treated in a single stage with endoscopic lysis and topical application of mitomycin C for prevention of anterior glottic restenosis. This case and prior reports suggest that the novel approach may be effective in the treatment of laryngeal webs.

Aortic insufficiency

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

Early results of valve-sparing ascending aortic replacement in type A aortic dissection and aortic insufficiency

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М. Л. Гордеев

2016-08-01

Full Text Available Aim: The study was designed to investigate predictors of effective valve-sparing ascending aortic replacement in patients with Stanford type A aortic dissection combined with aortic insufficiency and to analyze efficacy and safety of this kind of surgery.Methods: From January 2010 to December 2015, 49 patients with Stanford type A aortic dissection combined with aortic insufficiency underwent ascending aortic replacement. All patients were divided into 3 groups: valve-sparing procedures (group 1, n = 11, combined aortic valve and supracoronary ascending aortic replacement (group 2, n = 12, and Bentall procedure (group 3, n = 26. We assessed the initial status of patients, incidence of complications and efficacy of valve-sparing ascending aortic replacement.Results: The hospital mortality rate was 8.2% (4/49 patients. The amount of surgical correction correlated with the initial diameter of the aorta at the level of the sinuses of Valsalva. During the hospital period, none of patients from group 1 developed aortic insufficiency exceeding Grade 2 and the vast majority of patients had trivial aortic regurgitation. The parameters of cardiopulmonary bypass, cross-clamp time and circulatory arrest time did not correlate with the initial size of the ascending aorta and aortic valve blood flow impairment, neither did they influence significantly the incidence and severity of neurological complications. The baseline size of the ascending aorta and degree of aortic regurgitation did not impact the course of the early hospital period.Conclusions: Supracoronary ascending aortic replacement combined with aortic valve repair in ascending aortic dissection and aortic regurgitation is effective and safe. The initial size of the ascending aorta and aortic arch do not influence immediate results. The diameter of the aorta at the level of the sinuses of Valsalva and the condition of aortic valve leaflets could be considered as the limiting factors. Further long

Hybrid aortic repair with antegrade supra-aortic and renovisceral debranching from ascending aorta.

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Del Castro-Madrazo, José Antonio; Rivas-Domínguez, Margarita; Fernández-Prendes, Carlota; Zanabili Al-Sibbai, Amer; Llaneza-Coto, José Manuel; Alonso-Pérez, Manuel

2017-05-01

Aortic dissection is a life threatening condition. Hybrid repair has been described for the treatment of complex aortic pathology such as thoracoabdominal aortic aneurysms (TAAA) and type A and B dissections, although open and total endovascular repair are also possible. Open surgery is still associated with substantial perioperative morbi-mortality rates, thus less invasive techniques such as endovascular repair and hybrid procedures can achieve good results in centers with experience. We present the case of a patient with a chronic type B dissection and TAAA degeneration that was treated in a single stage hybrid procedure with antegrade supra-aortic and renovisceral debranching from the ascending aorta and TEVAR. At three-year follow up, the patient is free of intervention-related complications.

Abdominal aortic aneurysm development in men following a "normal" aortic ultrasound scan.

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Hafez, H; Druce, P S; Ashton, H A

2008-11-01

To determine predictors related to abdominal aortic aneurysm (AAA) development following a "normal" aortic ultrasound scan. Over a 23-year period, 22 961 men participated in an AAA screening programme. Maximum aortic diameter of less than 30 mm was deemed "normal". 4308 of these "normal" individuals were later re-scanned at intervals for research purposes. AAA prevalence was 4.4% at initial scanning. In those with a normal scan, 46 patients subsequently presented with AAAs incidentally detected and 120 (2.8%) had AAAs identified as part of the ongoing surveillance. The median initial aortic size of these 166 men was 25 mm (range 15-29 mm). Over the follow-up period, there have been 24 (14%) AAA-related deaths, 24 patients underwent successful AAA surgery and 36 died of unrelated causes. In those with an initial aortic diameter of <25 mm who later developed an AAA, the odds ratio for AAA-related mortality was 2 (95% CI 1-4.1, p=0.03, x(2)). AAAs can develop following an initial "normal" scan and men with an aortic diameters of 25-29 mm appear to be at greater risk. Surveillance for this sub-group may further reduce the incidence of undiagnosed AAA and AAA-related mortality.

Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos

International Nuclear Information System (INIS)

Zhang, Ting; Zhou, Xin-Ying; Ma, Xu-Fa; Liu, Jing-Xia

2015-01-01

Highlights: Using high-throughput in situ hybridization screening, we found that genes labeling the neural crest and its derivative pigment cells were sensitive to cadmium toxicity during zebrafish organogenesis, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in cadmium-exposed embryos. Based on neural crest markers, we identified the doses and times of cadmium exposure that cause damage to the zebrafish organogenesis, and we also found that compounds BIO or RA could neutralize the toxic effects of cadmium. - Abstract: Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd.

Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos

Energy Technology Data Exchange (ETDEWEB)

Zhang, Ting, E-mail: zting@webmail.hzau.edu.cn; Zhou, Xin-Ying, E-mail: 290356082@qq.com; Ma, Xu-Fa, E-mail: xufama@mail.hzau.edu.cn; Liu, Jing-Xia, E-mail: ichliu@mail.hzau.edu.cn

2015-10-15

Highlights: Using high-throughput in situ hybridization screening, we found that genes labeling the neural crest and its derivative pigment cells were sensitive to cadmium toxicity during zebrafish organogenesis, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in cadmium-exposed embryos. Based on neural crest markers, we identified the doses and times of cadmium exposure that cause damage to the zebrafish organogenesis, and we also found that compounds BIO or RA could neutralize the toxic effects of cadmium. - Abstract: Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd.

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

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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.

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

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

2006-09-20

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

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

International Nuclear Information System (INIS)

Yu, Jung Re; Ko, Seong Joo; Heo, Sang Taek; Kim, Jin Seok; Kim, Seung Hyoung

2013-01-01

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

Distraction osteogenesis of radiation-induced orbitozygomatic hypoplasia.

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Grover, Ramon; Murray, Dylan; Fialkov, Jeffrey A

2008-05-01

In the last decade, the application of distraction osteogenesis to the craniofacial skeleton has grown to include not only deformities of the mandible, but of the midface, palate, dentoalveolar region, and calvarium. A major advantage of distraction osteogenesis lies in the simultaneous soft tissue histogenesis that accompanies the bony distraction process, allowing for potentially lower relapse rates and improved cosmesis. Although this may seem appropriately suited to irradiation-induced deformities of both hard and soft tissues, there is little in the literature as to the efficacy of this technique in patients who have received radiotherapy. To introduce an effective application of this technology, and highlight some advantages and disadvantages of its application in the irradiated craniofacial skeleton, we present a case of distraction osteogenesis of the orbitozygomatic complex in a patient with radiation induced orbitozygomatic hypoplasia.

Aortic events in a nationwide Marfan syndrome cohort

DEFF Research Database (Denmark)

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

2017-01-01

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

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

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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.

Treatment of Severe Maxillary Hypoplasia With Combined Orthodontics and Distraction Osteogenesis.

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Lucchese, Alessandra; Albertini, Paolo; Asperio, Paolo; Manuelli, Maurizio; Gastaldi, Giorgio

2018-01-05

Distraction osteogenesis (DO) is a technique that allows the generation of new bone in a gap between 2 vascularized bone surfaces in response to the application of graduated tensile stress across the bone gap.Distraction osteogenesis has become a routine treatment of choice to correct skeletal deformities and severe bone defects in the craniofacial complex over the past decade. Distraction osteogenesis has been successfully chosen in lengthening the maxilla and the mandible; in the maxilla and recently in the mandible, the jawbones have been distracted and widened transversely to relieve severe anterior dental crowding and transverse discrepancies between the dental arches.Distraction osteogenesis for maxillary advancement started in 1993 and is now widely used, especially in patients with skeletal Class III malocclusion caused by maxillary hypoplasia.The aim of this study was to present the efficiency of combined orthodontic and DO in the severe maxillary hypoplasia.A 35-year-old Italian man presented to our clinical practice with the chief complaint of esthetic and functionally problems because of skeletal Class III malocclusion with anterior crossbite.Considering that the severity of the skeletal discrepancy is remarkable but compensated by the DO potential, the combined orthodontic and DO treatment was considered adequate, like less invasive and equally effective.It was obtained a good alignment with the upper and lower arch dental alveolar maxillary advancement that allowed to correct the sagittal relationships.The patient was satisfied for the treatment results and had considerable improvement in his self-esteem.

A Case of Fatal Pulmonary Hypoplasia with Congenital Diaphragmatic Hernia, Thoracic Myelomeningocele, and Thoracic Dysplasia.

Science.gov (United States)

Ito, Ai; Fujinaga, Hideshi; Matsui, Sachiko; Tago, Kumiko; Iwasaki, Yuka; Fujino, Shuhei; Nagasawa, Junko; Amari, Shoichiro; Kaneshige, Masao; Wada, Yuka; Takahashi, Shigehiro; Tsukamoto, Keiko; Miyazaki, Osamu; Yoshioka, Takako; Ishiguro, Akira; Ito, Yushi

2017-10-01

Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care. An autopsy revealed a left CDH without herniation of the liver or stomach into the thoracic cavity, severe hydrocephalus, Chiari malformation type II, MMC with spina bifida from Th4 to Th12, hemivertebrae, fused ribs, deformities of the thoracic cage and legs, short trunk, and agenesis of the left kidney. Conclusion  We speculate that two factors may be associated with the severe pulmonary hypoplasia: decreased thoracic space due to the herniation of visceral organs caused by CDH and thoracic dysplasia due to skeletal deformity and severe scoliosis.

MMP-2 Isoforms in Aortic Tissue and Serum of Patients with Ascending Aortic Aneurysms and Aortic Root Aneurysms

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Tscheuschler, Anke; Meffert, Philipp; Beyersdorf, Friedhelm; Heilmann, Claudia; Kocher, Nadja; Uffelmann, Xenia; Discher, Philipp; Siepe, Matthias; Kari, Fabian A.

2016-01-01

Objective The need for biological markers of aortic wall stress and risk of rupture or dissection of ascending aortic aneurysms is obvious. To date, wall stress cannot be related to a certain biological marker. We analyzed aortic tissue and serum for the presence of different MMP-2 isoforms to find a connection between serum and tissue MMP-2 and to evaluate the potential of different MMP-2 isoforms as markers of high wall stress. Methods Serum and aortic tissue from n = 24 patients and serum from n = 19 healthy controls was analyzed by ELISA and gelatin zymography. 24 patients had ascending aortic aneurysms, 10 of them also had aortic root aneurysms. Three patients had normally functioning valves, 12 had regurgitation alone, eight had regurgitation and stenosis and one had only stenosis. Patients had bicuspid and tricuspid aortic valves (9/15). Serum samples were taken preoperatively, and the aortic wall specimen collected during surgical aortic repair. Results Pro-MMP-2 was identified in all serum and tissue samples. Pro-MMP-2 was detected in all tissue and serum samples from patients with ascending aortic/aortic root aneurysms, irrespective of valve morphology or other clinical parameters and in serum from healthy controls. We also identified active MMP-2 in all tissue samples from patients with ascending aortic/aortic root aneurysms. None of the analyzed serum samples revealed signals relatable to active MMP-2. No correlation between aortic tissue total MMP-2 or tissue pro-MMP-2 or tissue active MMP-2 and serum MMP-2 was found and tissue MMP-2/pro-MMP-2/active MMP-2 did not correlate with aortic diameter. This evidence shows that pro-MMP-2 is the predominant MMP-2 species in serum of patients and healthy individuals and in aneurysmatic aortic tissue, irrespective of aortic valve configuration. Active MMP-2 species are either not released into systemic circulation or not detectable in serum. There is no reliable connection between aortic tissue—and serum MMP-2

Hypoplasia of bone marrow and cytopenia in non-hemic diseases

International Nuclear Information System (INIS)

Turbina, N.S.

1987-01-01

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

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

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

2003-01-01

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

Results of aortic root reimplantation in patients with ascending aortic aneurysm and concomitant aortic insufficiency

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А. М. Чернявский

2016-01-01

Full Text Available Objective. The research was designed to evaluate the results of valve-sparing operations: aortic root reimplantation versus aortic valve reimplantation when repairing an ascending aortic aneurysm with concomitant aortic insufficiency.Methods. Within a blind prospective randomized study conducted over a period from 2011 to 2015, 64 patients underwent aortic valve-sparing surgery. The inclusion criteria were the presence of an aortic aneurysm of the ascending aorta exceeding 4.5 cm and concomitant aortic insufficiency. All patients were divided into two groups: FS-group, aortic root reimplantation (modified Florida Sleeve technique (n = 32 and D-group, aortic valve reimplantation (David procedure (n = 32. The average age of patients was 57±13 (23–73 years in the FS-group and 55±11 (15–72 years in the D-group (p = 0.54. Both groups had 78% of males (p>0.99. A Marfan syndrome was identified in 6% and 9% in the FS-group and D-group respectively (p>0.99. Mean diameter of the sinuses of Valsalva was 51±7 mm and 56±10 mm (p = 0.09, aortic insufficiency 2.6±0.7 and 2.8±0.8 (p = 0.15 in the FS-group and D-group respectively. In the FS-group and D-group LVEDD amounted to 5.5±0.7 mm and 5.9±1.0 mm (p = 0.09 respectively. All patients took echocardiography in the preoperative, postoperative and follow-up periods.Results. In the long-term period, the degree of aortic regurgitation was 1.2±0.1 in the FS-group and 1.3±0.6 in the D-group (p = 0.72. LVEDD was 123±23 mm in the FS-group and 139.6±80 mm in the D-group at follow-up (p = 0.77. There were no statistically significant differences in the analysis of complications. Overall 30-day in-hospital mortality was 7.8%. There were 2 (6.3% deaths in the FS-group and 3 (9.4% in the D-group (p = 0.5.Late mortality was 6.3% in the FS-group and 3.1% in the D-group (p>0.99. Cumulative survival at 4 years was 84.3% and 84.8% in the FS-group and the D-group respectively (p = 0.94. Cumulative freedom from

Hypoplasia of the internal carotid artery with intercavernous anastomosis

International Nuclear Information System (INIS)

Chen, C.J.; Wang, L.J.; Wong, Y.C.; Chen, S.T.; Hsieh, F.Y.

1998-01-01

We report a symptomatic case of unilateral hypoplasia of the internal carotid artery with an intercavernous anastomosis, a very rare developmental anomaly. The symptoms were caused by occlusion of the proximal middle cerebral artery which possibly related to the haemodynamic stress caused by the anomalous intercavernous anastomosis. (orig.)

Treatment of enamel hypoplasia in a patient with Usher syndrome.

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de la Peña, Victor Alonso; Valea, Martín Caserío

2011-08-01

Usher syndrome (USH) is a group of autosomal recessive diseases characterized by the association of retinitis pigmentosa with sensorineural hearing loss. There are three types of USH. In addition, in people with USH and hypoplasia, the thickness of the enamel is reduced. The authors describe a case of a patient with USH type II associated with severe enamel hypoplasia and multiple unerupted teeth. The authors placed direct composite crowns and extracted severely affected and impacted molars. There is little information available on the oral pathologies of USH. Because the authors did not know how the patient's condition would progress and the patient still was growing, the authors treated the patient conservatively by placing direct composite crowns. The treatment has met both esthetic and functional expectations for 10 years. Copyright © 2011 American Dental Association. All rights reserved.

Application of thoracic endovascular aortic repair (TEVAR) in treating dwarfism with Stanford B aortic dissection: A case report.

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Qiu, Jian; Cai, Wenwu; Shu, Chang; Li, Ming; Xiong, Qinggen; Li, Quanming; Li, Xin

2018-04-01

To apply thoracic endovascular aortic repair (TEVAR) to treat dwarfism complicated with Stanford B aortic dissection. In this report, we presented a 63-year-old male patient of dwarfism complicated with Stanford B aortic dissection successfully treated with TEVAR. He was diagnosed with dwarfism complicated with Stanford B aortic dissection. After conservative treatment, the male patient underwent TEVAR at 1 week after hospitalization. After operation, he presented with numbness and weakness of his bilateral lower extremities, and these symptoms were significantly mitigated after effective treatment. At 1- and 3-week after TEVAR, the aorta status was maintained stable and restored. The patient obtained favorable clinical prognosis and was smoothly discharged. During subsequent follow-up, he remained physically stable. TEVAR is probably an option for treating dwarfism complicated with Stanford B aortic dissection, which remains to be validated by subsequent studies with larger sample size.

Aortic atresia with normal sized left ventricle

OpenAIRE

Priya Jagia; Arun Sharma; Saurabh K Gupta; Munish Guleria

2016-01-01

Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best ...

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

Science.gov (United States)

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

2014-02-01

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

Reoperative Aortic Root Replacement in Patients with Previous Aortic Root or Aortic Valve Procedures

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Byung Kwon Chong

2016-08-01

Full Text Available Background: Generalization of standardized surgical techniques to treat aortic valve (AV and aortic root diseases has benefited large numbers of patients. As a consequence of the proliferation of patients receiving aortic root surgeries, surgeons are more frequently challenged by reoperative aortic root procedures. The aim of this study was to evaluate the outcomes of redo-aortic root replacement (ARR. Methods: We retrospectively reviewed 66 patients (36 male; mean age, 44.5±9.5 years who underwent redo-ARR following AV or aortic root procedures between April 1995 and June 2015. Results: Emergency surgeries comprised 43.9% (n=29. Indications for the redo-ARR were aneurysm (n=12, pseudoaneurysm (n=1, or dissection (n=6 of the residual native aortic sinus in 19 patients (28.8%, native AV dysfunction in 8 patients (12.1%, structural dysfunction of an implanted bioprosthetic AV in 19 patients (28.8%, and infection of previously replaced AV or proximal aortic grafts in 30 patients (45.5%. There were 3 early deaths (4.5%. During follow- up (median, 54.65 months; quartile 1–3, 17.93 to 95.71 months, there were 14 late deaths (21.2%, and 9 valve-related complications including reoperation of the aortic root in 1 patient, infective endocarditis in 3 patients, and hemorrhagic events in 5 patients. Overall survival and event-free survival rates at 5 years were 81.5%±5.1% and 76.4%±5.4%, respectively. Conclusion: Despite technical challenges and a high rate of emergency conditions in patients requiring redo-ARR, early and late outcomes were acceptable in these patients.

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

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

2016-01-01

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

Aortic valve ochronosis: a rare manifestation of alkaptonuria.

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Steger, Christina Maria

2011-07-28

Alkaptonuric ochronosis is a heritable disorder of tyrosine metabolism, with various systemic abnormalities related to pigment deposition and degeneration of collagen and other tissues, including the heart and aorta. A 65-year-old woman with alkaptonuric ochronosis and a history of four joint replacements required aortic valve replacement for severe aortic stenosis. Operative findings included ochronosis of a partly calcified aortic valve and the aortic intima. The aortic valve was removed at surgery and histologically investigated. Light microscopic examination of the aortic valve revealed intracellular and extracellular deposits of ochronotic pigment and a chronic inflammatory infiltrate. Beside the case representation, the disease history, aetiology, pathogenesis, clinical presentation and treatment of aortic valve ochronosis are reviewed.

Hoarseness —As a presenting feature of aortic arch aneurysm

OpenAIRE

Mathai, John; Swapna, U. P.

2006-01-01

Hoarseness due to left recurrent laryngeal nerve paralysis (LRLN) caused by identifiable cardiovascular disease is described as Ortner’s syndrome or Cardiovocal syndrome. This was first described by Ortner in 1897 to describe left recurrent laryngeal nerve palsy secondary to Mitral Stenosis. The common conditions which gave rise to Ortners’ syndrome include mitral stenosis, mitral valve prolapse, aortic aneurysm, septal defect, following cardiothoracic surgery, high altitude pulmonary hyperte...

Wall stress on ascending thoracic aortic aneurysms with bicuspid compared with tricuspid aortic valve.

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Xuan, Yue; Wang, Zhongjie; Liu, Raymond; Haraldsson, Henrik; Hope, Michael D; Saloner, David A; Guccione, Julius M; Ge, Liang; Tseng, Elaine

2018-03-08

Guidelines for repair of bicuspid aortic valve-associated ascending thoracic aortic aneurysms have been changing, most recently to the same criteria as tricuspid aortic valve-ascending thoracic aortic aneurysms. Rupture/dissection occurs when wall stress exceeds wall strength. Recent studies suggest similar strength of bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms; thus, comparative wall stress may better predict dissection in bicuspid aortic valve versus tricuspid aortic valve-ascending thoracic aortic aneurysms. Our aim was to determine whether bicuspid aortic valve-ascending thoracic aortic aneurysms had higher wall stresses than their tricuspid aortic valve counterparts. Patients with bicuspid aortic valve- and tricuspid aortic valve-ascending thoracic aortic aneurysms (bicuspid aortic valve = 17, tricuspid aortic valve = 19) greater than 4.5 cm underwent electrocardiogram-gated computed tomography angiography. Patient-specific 3-dimensional geometry was reconstructed and loaded to systemic pressure after accounting for prestress geometry. Finite element analyses were performed using the LS-DYNA solver (LSTC Inc, Livermore, Calif) with user-defined fiber-embedded material model to determine ascending thoracic aortic aneurysm wall stress. Bicuspid aortic valve-ascending thoracic aortic aneurysms 99th-percentile longitudinal stresses were 280 kPa versus 242 kPa (P = .028) for tricuspid aortic valve-ascending thoracic aortic aneurysms in systole. These stresses did not correlate to diameter for bicuspid aortic valve-ascending thoracic aortic aneurysms (r = -0.004) but had better correlation to tricuspid aortic valve-ascending thoracic aortic aneurysms diameter (r = 0.677). Longitudinal stresses on sinotubular junction were significantly higher in bicuspid aortic valve-ascending thoracic aortic aneurysms than in tricuspid aortic valve-ascending thoracic aortic aneurysms (405 vs 329 kPa, P = .023). Bicuspid

Maxillary hypoplasia in the cleft patient: contribution of orthodontic dental space closure to orthognathic surgery.

Science.gov (United States)

Lee, Justine C; Slack, Ginger C; Walker, Ryann; Graves, Lindsay; Yen, Sandra; Woo, Jessica; Ambaram, Rishal; Martz, Martin G; Kawamoto, Henry K; Bradley, James P

2014-02-01

Cleft lip and palate surgery in the developing child is known to be associated with maxillary hypoplasia. However, the effects of nonsurgical manipulations on maxillary growth have not been well investigated. The authors present the contribution of orthodontic dental space closure with canine substitution to maxillary hypoplasia and the need for orthognathic surgery. Cleft lip/palate and cleft palate patients older than 15 years of age were reviewed for dental anomalies, orthodontic canine substitution, and Le Fort I advancement. Skeletal relationships of the maxilla to the skull base (SNA), mandible (ANB), and facial height were determined on lateral cephalograms. Logistic regression analyses were performed to estimate odds ratios. Ninety-five patients were reviewed (mean age, 18.1 years). In 65 patients with congenitally missing teeth, 55 percent with patent dental spaces required Le Fort I advancement. In contrast, 89 percent who underwent canine substitution required Le Fort I advancement (p = 0.004). Canine substitution is associated with a statistically significant increase in maxillary retrognathia when compared with dental space preservation on lateral cephalograms (mean SNA, 75.2 and 79.0, respectively; p = 0.006). Adjusting for missing dentition, logistic regression analyses demonstrated that canine substitution is an independent predictor for orthognathic surgery (OR, 6.47) and maxillary retrusion defined by SNA orthodontic cleft closure using canine substitution with maxillary hypoplasia and subsequent Le Fort I advancement, and suggest systematic criteria for management of cleft-related dental agenesis. Therapeutic, III.

Indirect veneer treatment of anterior maxillary teeth with enamel hypoplasia

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Devi Eka Juniarti

2010-09-01

Full Text Available Background: Nowadays, aesthetic rehabilitation becomes a necessity. It is affected by patient’s background, especially career, social and economic status. The aesthetic abnormality of anterior teeth i.e discoloration, malposition and malformation can affect patient’s appearance, especially during smile. These dental abnormalities, as a result, can decrease patient’s performance. Dental malformation, for instance, can be caused by developmental tooth defect, such as enamel hypoplasia. Enamel hypoplasia is a developmental defect caused by the lack of matrix amount which leads to thin and porous enamel. Enamel hypoplasia can also be caused by matrix calcification disturbance starting from the formation and development of enamel matrix causing defect and permanent changes which can occur on one or more tooth. Purpose: The aim of the study is to improve dental discoloration and tooth surface texture on anterior maxillary teeth with enamel hypoplasia by using indirect veneer with porcelain material. Case: A 20 years-old woman with enamel hypoplasia came to the Dental Hospital, Faculty of Dentistry Airlangga University. The patient wanted to improve her anterior maxillary teeth. It is clinically known that there were some opaque white spots (chalky spotted and porous on anterior teeth’s surface. Case management: Indirect veneer with porcelain material had been chosen as a restoration treatment which has excellent aesthetics and strength, and did not cause gingival irritation. As a result, the treatment could improve the confidence of the patient, and could also make their function normal. Conclusion: Indirect veneer is an effective treatment, which can improve patient’s appearance and self confidence.Latar belakang: Saat ini perbaikan estetik menjadi suatu kebutuhan. Kebutuhan akan estetik dipengaruhi latar belakang penderita, terutama karir, status sosial dan ekonomi. Hal ini disebabkan, kelainan estetik seperti diskolorasi, malposisi

Ascending aortic injuries following blunt trauma.

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Sun, Xiumei; Hong, Jenny; Lowery, Robert; Goldstein, Steven; Wang, Zuyue; Lindsay, Joseph; Hill, Peter C; Corso, Paul J

2013-11-01

The diagnosis and the management of traumatic thoracic aortic injuries have undergone significant changes due to new technology and improved prehospital care. Most of the discussions have focused on descending aortic injuries. In this review, we discuss the recent management of ascending aortic injuries. We found 5 cohort studies on traumatic aortic injuries and 11 case reports describing ascending aortic injuries between 1998 to the present through Medline research. Among case reports, 78.9% of cases were caused by motor vehicle accidents (MVA). 42.1% of patients underwent emergent open repair and the operative mortality was 12.5%. 36.8% underwent delayed repair. Associated injuries occurred in 84.2% of patients. Aortic valve injury was concurrent in 26.3% of patients. The incidence of ascending aortic injury ranged 1.9-20% in cohort studies. Traumatic injuries to the ascending aorta are relatively uncommon among survivors following blunt trauma. Aortography has been replaced by computed tomography and echocardiography as a diagnostic tool. Open repair, either emergent or delayed, remains the treatment of choice. © 2013 Wiley Periodicals, Inc.

Postoperative Reverse Remodeling and Symptomatic Improvement in Normal-Flow Low-Gradient Aortic Stenosis After Aortic Valve Replacement

DEFF Research Database (Denmark)

Carter-Storch, Rasmus; Møller, Jacob E; Christensen, Nicolaj L

2017-01-01

BACKGROUND: Severe aortic stenosis (AS) most often presents with reduced aortic valve area (benefit of aortic valve...... replacement (AVR) among NFLG patients is controversial. We compared the impact of NFLG condition on preoperative left ventricular (LV) remodeling and myocardial fibrosis and postoperative remodeling and symptomatic benefit. METHODS AND RESULTS: Eighty-seven consecutive patients with reduced aortic valve area...... and normal stroke volume index undergoing AVR underwent echocardiography, magnetic resonance imaging, a 6-minute walk test, and measurement of natriuretic peptides before and 1 year after AVR. Myocardial fibrosis was assessed from magnetic resonance imaging. Patients were stratified as NFLG or normal...

Alkaptonuria-associated aortic stenosis.

Science.gov (United States)

Lok, Zoe S Y; Goldstein, Jacob; Smith, Julian A

2013-07-01

Alkaptonuria is an autosomal recessive disorder of tyrosine metabolism, which results in accumulation of unmetabolized homogentisic acid and its oxidized product in various tissues, including the heart. Cardiovascular involvement is a rare but serious complication of the disease. We present two patients who have undergone successful aortic valve replacement for alkaptonuria-associated aortic stenosis along with a review of the literature. © 2013 Wiley Periodicals, Inc.

Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos.

Science.gov (United States)

Zhang, Ting; Zhou, Xin-Ying; Ma, Xu-Fa; Liu, Jing-Xia

2015-10-01

Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd. Copyright © 2015 Elsevier B.V. All rights reserved.

Aortic atresia with normal sized left ventricle

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Priya Jagia

2016-01-01

Full Text Available Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best of our knowledge, use of multi-detector computed tomography in aortic atresia with well developed left ventricle has not been reported in literature till date.

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

International Nuclear Information System (INIS)

Ilkko, E.; Tikkakoski, T.; Pyhtinen, J.

1998-01-01

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

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

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Karel Van Praet

2015-07-01

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

Aortic Root Enlargement or Sutureless Valve Implantation?

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Nikolaos G. Baikoussis

2016-11-01

Full Text Available Aortic valve replacement (AVR in patients with a small aortic annulus is a challenging issue. The importance of prosthesis–patient mismatch (PPM post aortic valve replacement (AVR is controversial but has to be avoided. Many studies support the fact that PPM has a negative impact on short and long term survival. In order to avoid PPM, aortic root enlargement may be performed. Alternatively and keeping in mind that often some comorbidities are present in old patients with small aortic root, the Perceval S suturelles valve implantation could be a perfect solution. The Perceval sutureless bioprosthesis provides reasonable hemodynamic performance avoiding the PPM and providing the maximum of aortic orifice area. We would like to see in the near future the role of the aortic root enlargement techniques in the era of surgical implantation of the sutureless valve (SAVR and the transcatheter valve implantation (TAVI.

Aortic valve bypass

DEFF Research Database (Denmark)

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

2013-01-01

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

Correction of aortic insufficiency with an external adjustable prosthetic aortic ring.

Science.gov (United States)

Gogbashian, Andrew; Ghanta, Ravi K; Umakanthan, Ramanan; Rangaraj, Aravind T; Laurence, Rita G; Fox, John A; Cohn, Lawrence H; Chen, Frederick Y

2007-09-01

Less invasive, valve-sparing options are needed for patients with aortic insufficiency (AI). We sought to evaluate the feasibility of reducing AI with an external adjustable aortic ring in an ovine model. To create AI, five sheep underwent patch plasty enlargement of the aortic annulus and root by placement of a 10 x 15 mm pericardial patch between the right and noncoronary cusps. An adjustable external ring composed of a nylon band was fabricated and placed around the aortic root. Aortic flow, aortic pressure, and left ventricular pressures were measured with the ring loose (off) and tightened (on). Mean regurgitant orifice area decreased by 86%, from 0.07 +/- 0.03 cm2 (ring loose, off) to 0.01 +/- 0.00 cm2 (ring tightened, on) [p < 0.01]. The regurgitant fraction decreased from 18 +/- 4% to 2 +/- 1% [p < 0.01]. The ring did not significantly affect stroke volume and aortic pressure. An ovine model of aortic root dilatation resulting in acute AI has been developed. In this model, application of an external, adjustable constricting aortic ring eliminated AI. An aortic ring may be a useful adjunct in reducing AI secondary to annular dilatation.

Association Between Gout and Aortic Stenosis.

Science.gov (United States)

Chang, Kevin; Yokose, Chio; Tenner, Craig; Oh, Cheongeun; Donnino, Robert; Choy-Shan, Alana; Pike, Virginia C; Shah, Binita D; Lorin, Jeffrey D; Krasnokutsky, Svetlana; Sedlis, Steven P; Pillinger, Michael H

2017-02-01

An independent association between gout and coronary artery disease is well established. The relationship between gout and valvular heart disease, however, is unclear. The aim of this study was to assess the association between gout and aortic stenosis. We performed a retrospective case-control study. Aortic stenosis cases were identified through a review of outpatient transthoracic echocardiography (TTE) reports. Age-matched controls were randomly selected from patients who had undergone TTE and did not have aortic stenosis. Charts were reviewed to identify diagnoses of gout and the earliest dates of gout and aortic stenosis diagnosis. Among 1085 patients who underwent TTE, 112 aortic stenosis cases were identified. Cases and nonaortic stenosis controls (n = 224) were similar in age and cardiovascular comorbidities. A history of gout was present in 21.4% (n = 24) of aortic stenosis subjects compared with 12.5% (n = 28) of controls (unadjusted odds ratio 1.90, 95% confidence interval 1.05-3.48, P = .038). Multivariate analysis retained significance only for gout (adjusted odds ratio 2.08, 95% confidence interval 1.00-4.32, P = .049). Among subjects with aortic stenosis and gout, gout diagnosis preceded aortic stenosis diagnosis by 5.8 ± 1.6 years. The age at onset of aortic stenosis was similar among patients with and without gout (78.7 ± 1.8 vs 75.8 ± 1.0 years old, P = .16). Aortic stenosis patients had a markedly higher prevalence of precedent gout than age-matched controls. Whether gout is a marker of, or a risk factor for, the development of aortic stenosis remains uncertain. Studies investigating the potential role of gout in the pathophysiology of aortic stenosis are warranted and could have therapeutic implications. Published by Elsevier Inc.

Pontoneocerebellar hypoplasia: definition of MR features

International Nuclear Information System (INIS)

Goasdoue, P.; Lalande, G.; Adamsbaum, C.; Rodriguez, D.; Moutard, M.-L.; Robain, O.

2001-01-01

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.)

Management of Cleft Maxillary Hypoplasia with Anterior Maxillary Distraction: Our Experience.

Science.gov (United States)

Chacko, Tojan; Vinod, Sankar; Mani, Varghese; George, Arun; Sivaprasad, K K

2014-12-01

Maxillary hypoplasia is a common developmental problem in cleft lip and palate deformities. Since 1970s these deformities have traditionally been corrected by means of orthognathic surgery. Management of skeletal deformities in the maxillofacial region has been an important challenge for maxillofacial surgeons and orthodontists. Distraction osteogenesis is a surgical technique that uses body's own repairing mechanisms for optimal reconstruction of the tissues. We present four cases of anterior maxillary distraction osteogenesis with tooth borne distraction device-Hyrax, which were analyzed retrospectively for the efficacy of the tooth borne device-Hyrax and skeletal stability of distracted anterior maxillary segment.

Intermittent, Non Cyclic Severe Mechanical Aortic Valve Regurgitation

Science.gov (United States)

Choi, Jong Hyun; Song, Seunghwan; Lee, Myung-Yong

2013-01-01

Mechanical aortic prosthesis dysfunction can result from thrombosis or pannus formation. We describe an unusual case of intermittent, non cyclic mechanical aortic prosthesis dysfunction due to pannus formation with thrombus in the absence of systolic restriction of disk excursion, that presented with intermittent severe aortic regurgitation. PMID:24459568

Aortic compliance in patients with aortic regurgitation. Evaluation with magnetic resonance imaging

International Nuclear Information System (INIS)

Murai, Sachiko; Hamada, Seiki; Ueguchi, Takashi

2005-01-01

The purpose of this study was to assess by means of cine magnetic resonance imaging (MRI) aortic compliance before and after aortic valve replacement (AVR with SJM (St. Jude Medical, St paul, MN) valve) in patients with aortic regurgitation (AR). Two groups (healthy controls and patients with severe isolated AR) of 10 subjects each were included in this study. Cine MRI was performed at three locations of the aorta, and aortic compliance was calculated by dividing the maximum change in the aortic area by pulse pressure. Cine MRI is useful to assess abnormalities of aortic compliance in patients with AR. Compared with the control group, aortic compliance in the AR group was significantly less in the ascending aorta (p<0.05), decreasing in order of aortic location. After AVR, aortic compliance improved for all locations. Cine MRI enables assessment of aortic biophysical properties such as a compliance for evaluating the progression of AR and the efficacy of treatment. (author)

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

Science.gov (United States)

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

2012-12-01

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

Aortic root replacement after previous surgical intervention on the aortic valve, aortic root, or ascending aorta.

Science.gov (United States)

Kirsch, E W Matthias; Radu, N Costin; Mekontso-Dessap, Armand; Hillion, Marie-Line; Loisance, Daniel

2006-03-01

Aortic root replacement after a previous operation on the aortic valve, aortic root, or ascending aorta remains a major challenge. Records of 56 consecutive patients (44 men; mean age, 56.4 +/- 13.6 years) undergoing reoperative aortic root replacement between June 1994 and June 2005 were reviewed retrospectively. Reoperation was performed 9.4 +/- 6.7 years after the last cardiac operation. Indications for reoperation were true aneurysm (n = 14 [25%]), false aneurysm (n = 10 [18%]), dissection or redissection (n = 9 [16%]), structural or nonstructural valve dysfunction (n = 10 [18%]), prosthetic valve-graft infection (n = 12 [21%]), and miscellaneous (n = 1 [2%]). Procedures performed were aortic root replacement (n = 47 [84%]), aortic root replacement plus mitral valve procedure (n = 5 [9%]), and aortic root replacement plus arch replacement (n = 4 [7%]). In 14 (25%) patients coronary artery bypass grafting had to be performed unexpectedly during the same procedure or immediately after the procedure to re-establish coronary perfusion. Hospital mortality reached 17.9% (n = 10). Multivariate logistic regression analysis revealed the need for unplanned perioperative coronary artery bypass grafting as the sole independent risk factor for hospital death (P = .005). Actuarial survival was 83.8% +/- 4.9% at 1 month, 73.0% +/- 6.3% at 1 year, and 65.7% +/- 9.0% at 5 years after the operation. One patient had recurrence of endocarditis 6.7 months after the operation and required repeated homograft aortic root replacement. Reoperative aortic root replacement remains associated with a high postoperative mortality. The need to perform unplanned coronary artery bypass grafting during reoperative aortic root replacement is a major risk factor for hospital death. The optimal technique for coronary reconstruction in this setting remains to be debated.

Type A aortic dissection associated with Dietzia maris.

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Reyes, Guillermo; Navarro, José-Luis; Gamallo, Carlos; delas Cuevas, María-Carmen

2006-10-01

Aortitis is a rare cause of aortic dissection. We report the unusual presentation of a 77-year-old male patient who underwent emergency surgery for an aortic dissection type A. A purulent pericardial fluid and inflammatory aorta were found after chest opening. Several samples were sent for analysis. The ascending aorta presented a mild dilatation with a large haematoma infiltrating the aortic root. The distal part of the ascending aorta seemed unaffected. The aortic rupture was found one centimetre above the non-coronary cusp. Aortic wall tissues were extremely fragile and with an inflammatory aspect. The patient died in the theatre room. In the histological study one out of three fragments of ascending aorta displayed longitudinal splitting of the outer media, with blood extravasation in the adventitial layer. In this level, the presence of a detritus material that reminded of bacterial colonies was noteworthy, together with abundant fibrinous exudates. In the laboratory a new specimen, Dietzia maris, was found in the pericardial liquid and in the aortic wall. We believe that this is the first reported finding of Dietzia maris in a patient with aortic disease.

Ruptured Abdominal Aortic Aneurysm: Prediction of Mortality From Clinical Presentation and Glasgow Aneurysm Score.

Science.gov (United States)

Weingarten, Toby N; Thompson, Lauren T; Licatino, Lauren K; Bailey, Christopher H; Schroeder, Darrell R; Sprung, Juraj

2016-04-01

To examine association of presenting clinical acuity and Glasgow Aneurysm Score (GAS) with perioperative and 1-year mortality. Retrospective chart review. Major tertiary care facility. Patients with ruptured abdominal aortic aneurysm (rAAA) from 2003 through 2013. Emergency repair of rAAA. The authors reviewed outcomes after stable versus unstable presentation and by GAS. Unstable presentation included hypotension, cardiac arrest, loss of consciousness, and preoperative tracheal intubation. In total, 125 patients (40 stable) underwent repair. Perioperative mortality rates were 41% and 12% in unstable and stable patients, respectively (pClinical presentation and GAS identified patients with rAAA who were likely to have a poor surgical outcome. GAS≥96 was associated with poor long-term survival, but>20% of these patients survived 1 year. Thus, neither clinical presentation nor GAS provided reliable guidance for decisions regarding futility of surgery. Copyright © 2016 Elsevier Inc. All rights reserved.

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

Science.gov (United States)

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

2017-08-01

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

Bilateral congenital absence of flexor pollicis longus with thumb hypoplasia and thenar atrophy

International Nuclear Information System (INIS)

Chaudhary, Vikas; Sehgal, Harsha; Bano, Shahina; Parmar, Pranjali R; Kumar, Sanjay

2014-01-01

Congenital absence of flexor pollicis longus with or without associated anomalies of thenar muscles and thumb is of rare occurrence. Inability to flex the interphalangeal joint of the thumb and absent dorsal wrinkles and flexion creases of the thumb are important clues to the diagnosis. Routine radiography and cross-sectional imaging help to confirm and document the condition. This article presents an extremely rare case of bilateral congenital absence of flexor pollicis longus tendon with thumb hypoplasia and thenar atrophy

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

International Nuclear Information System (INIS)

Ilkko, E.; Tikkakoski, T.; Pyhtinen, J.

1998-01-01

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.)

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

Severe hypoplasia of the omasal laminae in a Japanese Black steer with chronic bloat--a case report.

Science.gov (United States)

Takagi, Mitsuhiro; Mukai, Shuhei; Fushimi, Yasuo; Matsushita, Kouhei; Miyoshi, Nobuaki; Yasuda, Nobuhiro; Kitajima, Hideo; Takamure, Senro; Matsushita, Toshihiko; Kitamura, Nobuo; Deguchi, Eisaburo

2007-12-01

An 11-month-old Japanese Black steer with chronic bloat underwent clinical and histological analyses. During the observation period, it showed normal appetite and fecal volume but persistent chronic bloat symptoms. Compared to controls, the steer's feces contained undigested large straws. Necropsy revealed normal rumen, reticulum, and abomasum but a small omasum. The rumen, reticulum, and abomasum mucosa was normal, with well-developed ruminal papillae. However, severe hypoplasia of the omasal laminae was observed along with hypoplasia reticular groove and ruminoreticular fold. The contents of the reticulum, omasum, and abomasums comprised undigested large sized hay particles. The omasum papillae showed no pathological abnormalities. This is a rare case of a steer with chronic bloat probably caused by severe hypoplasia of the omasal laminae.

Maxillary Hypoplasia With Congenital Oligodontia Treated by Maxillary Distraction Osteogenesis.

Science.gov (United States)

Mishima, Sayaka; Yamaguchi, Takako; Watanabe, Takuma; Komatani, Toru; Nakao, Kazumasa; Takahashi, Katsu; Bessho, Kazuhisa

2018-02-27

It is known that congenitally missing teeth can often cause differences in craniofacial morphology; however, there are few reported cases of orthognathic surgical treatment for these patients. Herein, the authors report a rare case of maxillary hypoplasia with congenital oligodontia treated by maxillary distraction osteogenesis with internal device. A 17-year-old male presenting with multiple tooth agenesis and maxillary recession was referred to our hospital for orthognathic surgical treatment. Preoperative simulation surgery was performed using Full-Color 3-dimensional salt model. After surgery, improvement in maxillary recession and occlusal stability was observed. This report demonstrates the advantages of the method used herein, which includes reduction in operating time with increase in the safety of the procedure.

The future of aortic surgery in Europe

DEFF Research Database (Denmark)

Czerny, Martin; Bachet, Jean; Bavaria, Joseph

2012-01-01

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

Congenital bicuspid stenosis with left ventricular hypoplasia in a kitten.

Science.gov (United States)

van Nie, C J; van Messel, M A; Straatman, T J

1980-01-15

Congenital bicuspid stenosis with left ventricular hypoplasia was diagnosed in a kitten. Clinical weakness, dyspnoea and marked cardiomegaly (X rays) were related to postmortem findings. The cardiomegaly had resulted from an enlargement of the left auricular appendage. It is supposed the cardiomegaly developed after the closing of the foramen ovale.

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

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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.

Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: A case report and review of literature.

Science.gov (United States)

Sarhan, Osama M; Al-Ghanbar, Mustafa S; Nakshabandi, Ziad M

2013-10-01

Association between Prune belly syndrome (PBS) and urethral hypoplasia is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypoplasia and congenital vesico-cutaneous fistula in a male neonate. Patient underwent cutaneous vesicostomy and was discharged for close follow up of his renal function and for future reconstruction.

Short limbed dwarfism, genital hypoplasia, sparse hair, and vertebral anomalies: a variant of Ellis-van Creveld syndrome?

OpenAIRE

Fryns, J P; Moerman, P

1993-01-01

A male newborn with acromesomelic short limbed dwarfism, genital hypoplasia, and vertebral anomalies is reported. As the child had an important number of clinical and radiological symptoms seen in patients with Ellis-van Creveld syndrome, we raise the question of whether he may represent a variant example of this syndrome despite the absence of cardinal symptoms such as postaxial polydactyly and ectodermal changes (nail hypoplasia).

The bicuspid aortic valve and its relation to aortic dilation

Directory of Open Access Journals (Sweden)

Shi-Min Yuan

2010-01-01

Full Text Available BACKGROUND: A bicuspid aortic valve (BAV is a common congenital heart disease, which affects 1-2% of the population. However, the relationship between BAVs and aortic dilation has not been sufficiently elucidated. METHODS: A total of 241 BAV patients who were referred to this hospital for cardiac surgey over a 4.75-year period were included in this study. In addition to the clinical characteristics of the included patients, the morphological features of the aortic valve and aorta, the length of the left main coronary artery, and the laboratory findings (the coagulation and hematological parameters as well as the total cholesterol concentration were determined and compared with those of the tricuspid aortic valve (TAV patients. RESULTS: The BAV patients were younger than the TAV patients for a valve surgery in the last 3 months of the study period. The BAV patients were predominantly male. Most of the BAVs that were surgically treated were stenotic, regurgitant, or combined, and only 19 (7.88% were normally functioning valves. According to echocardiography or operative records, 148 (78.31% were type A, 31 (16.40% were type B, and 10 (5.29% were type C. The left main coronary artery was much shorter in the BAV patients than it was in the TAV patients. There was no significant difference between BAV and TAV patients in the total cholesterol concentrations; whereas differences were noted between patients receiving lipid-lowering therapy and those not receiving lipid-lowering therapy. The dimensions of the aortic root, sinotubular junction, and ascending aorta were beyond normal limits, while they were significantly smaller in the BAV patients than in the TAV patients. They were also much smaller in patients receiving statin therapy than those not receiving statin therapy in both groups. Moreover, the aortic dilation in the BAV group was found to be significantly associated with patient age. CONCLUSIONS: The BAV patients developed aortic wall and

Aortic root reimplantation procedure: a new milestone in aortic valve-sparing operations

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А. М. Чернявский

2016-08-01

Full Text Available Aim: Emphasis in this study was placed on clinical and functional assessment of a modified "Florida Sleeve" procedure during surgical correction of ascending aorta aneurysms with concomitant aortic insufficiency.Methods: 32 patients with an aneurysm of the ascending aorta and aortic insufficiency underwent a modified "Florida Sleeve" procedure. The average follow-up was 17 (0-60 months. The average age of patients was 57±13 (23-73 years 56±13 years.Results: The expected 4-year cumulative survival rate was 84.3%. Overall freedom from aortic insufficiency in the late period was 88.9%. Median aortic regurgitation was 1+ (1; 2. Long-term follow-up revealed no valve-associated complications.Conclusion: The aortic root reimplantation procedure enables optimal correction of the existing lesions of the aortic root without performing aortic valve replacement and demonstrates stable clinical and functional outcomes in the long-term period.Key words: aortic aneurysm; aortic valve; valve-sparing operations.FundingThe study had no sponsorship.Conflict of interestThe authors declare no conflict of interest.

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

OpenAIRE

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

2012-01-01

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

Aortic valve replacement for aortic stenosis caused by alkaptonuria.

Science.gov (United States)

Hiroyoshi, Junko; Saito, Aya; Panthee, Nirmal; Imai, Yasushi; Kawashima, Dai; Motomura, Noboru; Ono, Minoru

2013-03-01

We report a case of aortic stenosis associated with ochronosis in a 70-year-old man who underwent biologic aortic valve replacement. Intraoperative findings included ochronosis of a severely calcified pigmented aortic valve along with pigmentation of the intima of the aorta. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Shape of the dilated aorta in children with bicuspid aortic valve

International Nuclear Information System (INIS)

Mart, Christopher R; McNerny, Bryn E

2013-01-01

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

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

Science.gov (United States)

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

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

Science.gov (United States)

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

2018-01-29

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

Mechanisms of recurrent aortic regurgitation after aortic valve repair: predictive value of intraoperative transesophageal echocardiography.

Science.gov (United States)

le Polain de Waroux, Jean-Benoît; Pouleur, Anne-Catherine; Robert, Annie; Pasquet, Agnès; Gerber, Bernhard L; Noirhomme, Philippe; El Khoury, Gébrine; Vanoverschelde, Jean-Louis J

2009-08-01

The aim of the present study was to examine the intraoperative echocardiographic features associated with recurrent severe aortic regurgitation (AR) after an aortic valve repair surgery. Surgical valve repair for AR has significant advantages over valve replacement, but little is known about the predictors and mechanisms of its failure. We blindly reviewed all clinical, pre-operative, intraoperative, and follow-up transesophageal echocardiographic data of 186 consecutive patients who underwent valve repair for AR during a 10-year period and in whom intraoperative and follow-up echo data were available. After a median follow-up duration of 18 months, 41 patients had recurrent 3+ AR, 23 patients presented with residual 1+ to 2+ AR, and 122 had no or trivial AR. In patients with recurrent 3+ AR, the cause of recurrent AR was the rupture of a pericardial patch in 3 patients, a residual cusp prolapse in 26 patients, a restrictive cusp motion in 9 patients, an aortic dissection in 2 patients, and an infective endocarditis in 1 patient. Pre-operatively, all 3 groups were similar for aortic root dimensions and prevalence of bicuspid valve (overall 37%). Patients with recurrent AR were more likely to display Marfan syndrome or type 3 dysfunction pre-operatively. At the opposite end, patients with continent AR repair at follow-up were more likely to have type 2 dysfunction pre-operatively. After cardiopulmonary bypass, a shorter coaptation length, the degree of cusp billowing, a lower level of coaptation (relative to the annulus), a larger diameter of the aortic annulus and the sino-tubular junction, the presence of a residual AR, and the width of its vena contracta were associated with the presence of AR at follow-up. Multivariate Cox analysis identified a shorter coaptation length (odds ratio [OR]: 0.8, p = 0.05), a coaptation occurring below the level of the aortic annulus (OR: 7.9, p < 0.01), a larger aortic annulus (OR: 1.2, p = 0.01), and residual aortic regurgitation

Cerebral white matter hypoplasia

International Nuclear Information System (INIS)

Dietrich, R.B.; Shields, W.D.; Sankar, R.

1990-01-01

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

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

DEFF Research Database (Denmark)

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

2017-01-01

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

Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: A case report and review of literature

Directory of Open Access Journals (Sweden)

Osama M Sarhan

2013-01-01

Full Text Available Association between Prune belly syndrome (PBS and urethral hypoplasia is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypoplasia and congenital vesico-cutaneous fistula in a male neonate. Patient underwent cutaneous vesicostomy and was discharged for close follow up of his renal function and for future reconstruction.

Short limbed dwarfism, genital hypoplasia, sparse hair, and vertebral anomalies: a variant of Ellis-van Creveld syndrome?

Science.gov (United States)

Fryns, J P; Moerman, P

1993-01-01

A male newborn with acromesomelic short limbed dwarfism, genital hypoplasia, and vertebral anomalies is reported. As the child had an important number of clinical and radiological symptoms seen in patients with Ellis-van Creveld syndrome, we raise the question of whether he may represent a variant example of this syndrome despite the absence of cardinal symptoms such as postaxial polydactyly and ectodermal changes (nail hypoplasia). Images PMID:8487282

Generalized seizures in the right hippocampus sclerosis combined with hypoplasia of the right vertebral artery

International Nuclear Information System (INIS)

Manchev, L.; Toneva, J.; Manolova, T.; Manchev, I.; Valcheva, V.

2016-01-01

We present a clinical case of generalized epileptic seizures, occurring suddenly. The common finding from MRI of the brain is sclerosis of the right hippocampus, while MR angiography shows hypoplasia of the right vertebral artery. There are EEG signs for single foci of abnormal activity more on the right side. An anticonvulsant and symptomatic treatment demonstrate a favorable result. Under discussion is the question of surgery treatment. Key words: Hippocampal Sclerosis. MRI. Epileptic Seizures

Impact of bicuspid aortic valve on complications and death in infective endocarditis of native aortic valves.

Science.gov (United States)

Kahveci, Gokhan; Bayrak, Fatih; Pala, Selcuk; Mutlu, Bulent

2009-01-01

We retrospectively investigated the impact of bicuspid aortic valve on the prognosis of patients who had definite infective endocarditis of the native aortic valve.Of 51 patients, a bicuspid aortic valve was present in 22 (43%); the other 29 had tricuspid aortic valves. On average, the patients who had bicuspid valves were younger than those who had tricuspid valves. Patients with a tricuspid valve had larger left atrial diameters and were more likely to have severe mitral regurgitation.Periannular complications, which we detected in 19 patients (37%), were much more common in the patients who had a bicuspid valve (64% vs 17%, P = 0.001). The presence of a bicuspid valve was the only significant independent predictor of periannular complications. The in-hospital mortality rate in the bicuspid group was lower than that in the tricuspid group; however, this figure did not reach statistical significance (9% vs 24%, P = 0.15). In multivariate analysis, left atrial diameter was the only independent predictor associated with an increased risk of death (hazard ratio, 2.19; 95% confidence interval, 1.1-4.5; P = 0.031).In our study, patients with infective endocarditis in a bicuspid aortic valve were younger and had a higher incidence of periannular complications. Although a worse prognosis has been reported previously, we found that infective endocarditis in a native bicuspid aortic valve is not likely to increase the risk of death in comparison with infective endocarditis in native tricuspid aortic valves.

The use of transcatheter aortic valve replacement vs surgical aortic valve replacement for the treatment of aortic stenosis

Directory of Open Access Journals (Sweden)

Jensen HA

2015-08-01

Full Text Available Hanna A Jensen, Lillian L Tsai, Vinod H Thourani Division of Cardiothoracic Surgery, Joseph B Whitehead Department of Surgery, Structural Heart and Valve Center, Emory University School of Medicine, Atlanta, GA, USA Abstract: Severe aortic stenosis (AS is associated with considerable morbidity and mortality and is increasing in prevalence as the global population increases. Since AS primarily affects the elderly, many of these patients have comorbidities that make them poor candidates for the gold standard treatment for AS, surgical aortic valve replacement. Transcatheter aortic valve replacement has emerged as a novel technology for the management of AS in higher risk patients over the past decade. Randomized trials have established the safety and efficacy of transcatheter aortic valve replacement, and the medical community has rallied to identify the patients who are most suitable for this transformative treatment. This review focuses on outlining the key procedural differences, describing the unique challenges of both operations, and finally assessing and comparing outcomes both on a general level and in challenging patient subgroups. Keywords: aortic valve replacement, transcatheter aortic valve replacement, surgical aortic valve replacement 

Altered aortic shape in bicuspid aortic valve relatives influences blood flow patterns.

Science.gov (United States)

Schnell, Susanne; Smith, Danielle A; Barker, Alex J; Entezari, Pegah; Honarmand, Amir R; Carr, Maria L; Malaisrie, S Chris; McCarthy, Patrick M; Collins, Jeremy; Carr, James C; Markl, Michael

2016-11-01

Bicuspid aortic valve (BAV) is known to exhibit familial inheritance and is associated with aortopathy and altered aortic haemodynamics. However, it remains unclear whether BAV-related aortopathy can be inherited independently of valve morphology. Four-dimensional flow magnetic resonance imaging for the in vivo assessment of thoracic aortic 3D blood flow was performed in 24 BAV relatives with trileaflet aortic valves (age = 40 ± 14 years) and 15 healthy controls (age = 37 ± 10 years). Data analysis included aortic dimensions, shape (round/gothic/cubic), and 3D blood flow characteristics (semi-quantitative vortex/helix grading and peak velocities). Cubic and gothic aortic shapes were markedly more prevalent in BAV relatives compared with controls (38 vs. 7%). Ascending aorta (AAo) vortex flow in BAV relatives was significantly increased compared with controls (grading = 1.5 ± 1.0 vs. 0.6 ± 0.9, P = 0.015). Aortic haemodynamics were influenced by aortic shape: peak velocities were reduced for gothic aortas vs. round aortas (P = 0.003); vortex flow was increased for cubic aortas in the AAo (P gothic aortas in the AAo and descending aorta (P = 0.003, P = 0.029). Logistic regression demonstrated significant associations of shape with severity of vortex flow in AAo (P < 0.001) and aortic arch (P = 0.016) in BAV relatives. BAV relatives expressed altered aortic shape and increased vortex flow despite the absence of valvular disease or aortic dilatation. These data suggest a heritable component of BAV-related aortopathy affecting aortic shape and aberrant blood flow, independent of valve morphology. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.

Aortic events in a nationwide Marfan syndrome cohort.

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

2017-02-01

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

Intermittent, noncyclic dysfunction of a mechanical aortic prosthesis by pannus formation.

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Giroux, Sylvie K; Labinaz, Marino X; Grisoli, Dominique; Klug, Andrew P; Veinot, John P; Burwash, Ian G

2010-01-01

Mechanical aortic prosthesis dysfunction can result from thrombosis or pannus formation. Pannus formation usually restricts systolic excursion of the occluding disk, resulting in progressive stenosis of the aortic prosthesis. Intermittent dysfunction of a mechanical aortic prosthesis is usually ascribed to thrombus formation. We describe an unusual case of intermittent, noncyclic dysfunction of a mechanical aortic prosthesis due to pannus formation in the absence of systolic restriction of disk excursion that presented with intermittent massive aortic regurgitation, severe ischemia, and shock. Pannus formation should be considered as a potential cause of acute intermittent severe aortic regurgitation in a patient with a mechanical aortic prosthesis.

Full-root aortic valve replacement with stentless xenograft achieves superior regression of left ventricular hypertrophy compared to pericardial stented aortic valves.

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Tavakoli, Reza; Auf der Maur, Christoph; Mueller, Xavier; Schläpfer, Reinhard; Jamshidi, Peiman; Daubeuf, François; Frossard, Nelly

2015-02-03

Full-root aortic valve replacement with stentless xenografts has potentially superior hemodynamic performance compared to stented valves. However, a number of cardiac surgeons are reluctant to transform a classical stented aortic valve replacement into a technically more demanding full-root stentless aortic valve replacement. Here we describe our technique of full-root stentless aortic xenograft implantation and compare the early clinical and midterm hemodynamic outcomes to those after aortic valve replacement with stented valves. We retrospectively compared the pre-operative characteristics of 180 consecutive patients who underwent full-root replacement with stentless aortic xenografts with those of 80 patients undergoing aortic valve replacement with stented valves. In subgroups presenting with aortic stenosis, we further analyzed the intra-operative data, early postoperative outcomes and mid-term regression of left ventricular mass index. Patients in the stentless group were younger (62.6 ± 13 vs. 70.3 ± 11.8 years, p regression of the left ventricular mass index in the stentless (p replacement can be performed without adversely affecting the early morbidity or mortality in patients operated on for aortic valve stenosis provided that the coronary ostia are not heavily calcified. The additional time necessary for the full-root stentless compared to the classical stented aortic valve replacement is therefore not detrimental to the early clinical outcomes and is largely rewarded in patients with aortic stenosis by lower transvalvular gradients at mid-term and a better regression of their left ventricular mass index.

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

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

2018-02-01

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

Atypical Presentation of a Type 2 Endoleak following Emergency Open Repair of a Ruptured Abdominal Aortic Aneurysm

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K. Sharma

Full Text Available : Background: An endoleak is a common complication following EVAR. Specifically, a Type 2 endoleak occurs because of retrograde flow from lumbar vessels outside the endograft within the aneurysm sac. Even though it is common following EVAR, it has not been identified as a complication following open ruptured abdominal aortic aneurysm (AAA repair. Report: A 73-year-old male underwent open repair of a ruptured AAA. Five months later, computed tomography revealed filling from a lumbar vessel mimicking a Type 2 “endoleak.” The initial ultrasound showed a single pair of lumbar vessels with aneurysm sac expansion 8 weeks later. The “endoleak” and expanding sac were treated, and the 2-year surveillance demonstrated sac shrinkage. Discussion: Because endoleak is a complication after EVAR, this case provides a unique presentation of Type 2 “endoleak” physiology following open repair of a ruptured AAA. It is believed that it is necessary to expand the list of possible complications after open ruptured AAA repair to include “endoleaks.” Keywords: Endoleak: EVAR, Ruptured aneurysm, Open abdominal aortic aneurysm

Posterior communicating artery hypoplasia as a risk factor for acute ischemic stroke in the absence of carotid artery occlusion.

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Chuang, Yu-Ming; Liu, Chih-Yang; Pan, Po-Jung; Lin, Ching-Po

2008-12-01

Posterior communicating artery (PCoA) hypoplasia is a fetal variant of the Circle of Willis. According to angiograms and autopsy reports, this congenital variation is found in 6-21% of the general population. PCoA hypoplasia only becomes a risk factor for ischemic stroke in the presence of ipsilateral internal carotid artery (ICA) occlusion. The aim of our study was to determine the role of PCoA hypoplasia in acute ischemic stroke in the absence of ICA occlusion. We examined 310 acute ischemic stroke patients (mean age+/-standard deviation; 68.9+/-15.6 years). Cerebral magnetic resonance angiography was performed within 72 hours of ischemic stroke onset. For comparison, a risk factor-matched control group was recruited. Conditional logistic regression was used to calculate odds ratios (OR) and 95% confidence intervals (CI) to estimate the independent effect of potential risk factors. The overall incidence of PCoA hypoplasia in our experimental group was 19.35% (n=60), which was significantly higher than in the control group (8.20%, n=22, p=0.036, OR, 3.21; 95% CI, 1.43-9.62). The most common ischemic event was ipsilateral thalamic lacunar infarctions with or without occipital lobe involvement. Based on our results, PCoA hypoplasia appears to be a contributor to the risk of ischemic stroke, even in the absence of ICA occlusion. This risk is especially pronounced for strokes involving arteries that penetrate the thalamus.

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

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

2015-06-09

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

Aortic Baroreceptors Display Higher Mechanosensitivity than Carotid Baroreceptors

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Eva On-Chai Lau

2016-08-01

Full Text Available Arterial baroreceptors are mechanical sensors that detect blood pressure changes. It has long been suggested that the two arterial baroreceptors, aortic and carotid baroreceptors, have different pressure sensitivities. However, there is no consensus as to which of the arterial baroreceptors are more sensitive to changes in blood pressure. In the present study, we employed independent methods to compare the pressure sensitivity of the two arterial baroreceptors. Firstly, pressure-activated action potential firing was measured by whole-cell current clamp with a high-speed pressure clamp system in primary cultured baroreceptor neurons. The results show that aortic depressor neurons possessed a higher percentage of mechano-sensitive neurons. Furthermore, aortic baroreceptor neurons show a lower pressure threshold than that of carotid baroreceptor neurons. Secondly, uniaxial stretching of baroreceptor neurons, that mimics the forces exerted on blood vessels, elicited a larger increase in intracellular Ca2+ rise in aortic baroreceptor neurons than in carotid baroreceptor neurons. Thirdly, the pressure-induced action potential firing in the aortic depressor nerve recorded in vivo was also higher. The present study therefore provides for a basic physiological understanding on the pressure sensitivity of the two baroreceptor neurons and suggests that aortic baroreceptors have a higher pressure sensitivity than carotid baroreceptors.

Analysis of aortic root surgery with composite mechanical aortic valve conduit and valve-sparing reconstruction.

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Dias, Ricardo Ribeiro; Mejia, Omar Asdrubal Vilca; Fiorelli, Alfredo Inácio; Pomerantzeff, Pablo Maria Alberto; Dias, Altamiro Ribeiro; Mady, Charles; Stolf, Noedir Antonio Groppo

2010-01-01

Comparative analysis of early and late results of aortic root reconstruction with aortic valve sparing operations and the composite mechanical valve conduit replacement. From November 2002 to September 2009, 164 consecutive patients with mean age 54 ± 15 years, 115 male, underwent the aortic root reconstruction (125 mechanical valve conduit replacements and 39 valve sparing operations). Sixteen percent of patients had Marfan syndrome and 4.3% had bicuspid aortic valve. One hundred and forty-four patients (88%) were followed for a mean period of 41.1 ± 20.8 months. The hospital mortality was 4.9%, 5.6% in operations with valved conduits and 2.6% in the valve sparing procedures (P valve sparing operations, respectively (95% CI = 70% - 95%, P = 0.001), (95% CI = 82% - 95% P = 0.03) and (95% CI = 81% - 95%, P = 0.03). Multivariate analysis showed that creatinine greater than 1.4 mg/dl, Cabrol operation and renal dialysis were predictors of mortality, respectively, with occurrence chance of 6 (95% CI = 1.8 - 19.5, P = 0.003), 12 (95% CI = 3 - 49.7, P = 0.0004) and 16 (95% CI = 3.6 - 71.3, P = 0.0002). The aortic root reconstruction has a low early and late mortality, high survival free of complications and low need for reoperation. During the late follow-up, valve sparing aortic root reconstructions presented fewer incidences of bleeding, thromboembolic events and endocarditis.

Aortic stenosis and vascular calcifications in alkaptonuria.

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Hannoush, Hwaida; Introne, Wendy J; Chen, Marcus Y; Lee, Sook-Jin; O'Brien, Kevin; Suwannarat, Pim; Kayser, Michael A; Gahl, William A; Sachdev, Vandana

2012-02-01

Alkaptonuria is a rare metabolic disorder of tyrosine catabolism in which homogentisic acid (HGA) accumulates and is deposited throughout the spine, large joints, cardiovascular system, and various tissues throughout the body. In the cardiovascular system, pigment deposition has been described in the heart valves, endocardium, pericardium, aortic intima and coronary arteries. The prevalence of cardiovascular disease in patients with alkaptonuria varies in previous reports. We present a series of 76 consecutive adult patients with alkaptonuria who underwent transthoracic echocardiography between 2000 and 2009. A subgroup of 40 patients enrolled in a treatment study underwent non-contrast CT scans and these were assessed for vascular calcifications. Six of the 76 patients had aortic valve replacement. In the remaining 70 patients, 12 patients had aortic sclerosis and 7 patients had aortic stenosis. Unlike degenerative aortic valve disease, we found no correlation with standard cardiac risk factors. There was a modest association between the severity of aortic valve disease and joint involvement, however, we saw no correlation with urine HGA levels. Vascular calcifications were seen in the coronaries, cardiac valves, aortic root, descending aorta and iliac arteries. These findings suggest an important role for echocardiographic screening of alkaptonuria patients to detect valvular heart disease and cardiac CT to detect coronary artery calcifications. Published by Elsevier Inc.

Aortic valve-sparing surgery in Marfan syndrome.

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

2013-08-01

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

Acute aortic syndromes: definition, prognosis and treatment options.

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Carpenter, S W; Kodolitsch, Y V; Debus, E S; Wipper, S; Tsilimparis, N; Larena-Avellaneda, A; Diener, H; Kölbel, T

2014-04-01

Acute aortic syndromes (AAS) are life-threatening vascular conditions of the thoracic aorta presenting with acute pain as the leading symptom in most cases. The incidence is approximately 3-5/100,000 in western countries with increase during the past decades. Clinical suspicion for AAS requires immediate confirmation with advanced imaging modalities. Initial management of AAS addresses avoidance of progression by immediate medical therapy to reduce aortic shear stress. Proximal symptomatic lesions with involvement of the ascending aorta are surgically treated in the acute setting, whereas acute uncomplicated distal dissection should be treated by medical therapy in the acute period, followed by surveillance and repeated imaging studies. Acute complicated distal dissection requires urgent invasive treatment and thoracic endovascular aortic repair has become the treatment modality of choice because of favorable outcomes compared to open surgical repair. Intramural hematoma, penetrating aortic ulcers, and traumatic aortic injuries of the descending aorta harbor specific challenges compared to aortic dissection and treatment strategies are not as uniformly defined as in aortic dissection. Moreover these lesions have a different prognosis. Once the acute period of aortic syndrome has been survived, a lifelong medical treatment and close surveillance with repeated imaging studies is essential to detect impending complications which might need invasive treatment within the short-, mid- or long-term.

The use of internal maxillary distraction for maxillary hypoplasia: a preliminary report.

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Van Sickels, Joseph E; Madsen, Mathew J; Cunningham, Larry L; Bird, Douglas

2006-12-01

Distraction osteogenesis is a useful alternative to advance the maxilla in complicated cases of maxillary hypoplasia. The purpose of this article is to review the workup, experience, and preliminary results with the use of internal distraction osteogenesis for maxillary hypoplasia at one teaching institution. Over a 5-year period, more than 300 patients with craniofacial and dentofacial defects have undergone oral and maxillofacial surgery at our center to correct their skeletal discrepancies. Of these, 10 have had maxillary distraction osteogenesis done with internal distractors. Follow-up of 6 months or more was available for 8 patients. Stereolithographic models were used to bend distractors prior to surgery in 6 patients. Latency prior to the start of distraction was 3 to 7 days and varied with the age of the patient. Distraction occurred at approximately 1 mm per day with an average distraction length of 8.5 mm (range, 6-10 mm). Excellent occlusal results were obtained in 5 patients. Major complications including nonunion and failure to achieve acceptable occlusal results were observed in 3 patients. Minor complications including pain and loosening of the distracter devices were observed in 2 patients, but did not appear to affect the esthetic and functional results. Distraction osteogenesis is a useful alternative to traditional orthognathic surgery to treat maxillary hypoplasia. Internal distractions are attractive to patients, but are more difficult to place and can cause discomfort to patients when trying to achieve an ideal primary vector of distraction. Stereolithographic models can help with placement of the device. Changes in design of distractors may help with patient discomfort.

Clinical and molecular diagnosis of a cartilage-hair hypoplasia with IGF-1 deficiency.

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Castilla-Cortázar, Inma; Rodríguez De Ita, Julieta; Martín-Estal, Irene; Castorena, Fabiola; Aguirre, Gabriel A; García de la Garza, Rocío; Elizondo, Martha I

2017-02-01

Cartilage-hair hypoplasia syndrome (CHH) is a rare autosomal recessive condition characterized by metaphyseal chondrodysplasia and characteristic hair, together with a myriad of other symptoms, being most common immunodeficiency and gastrointestinal complications. A 15-year-old Mexican male initially diagnosed with Hirschsprung disease and posterior immunodeficiency, presents to our department for genetic and complementary evaluation for suspected CHH. Physical, biochemical, and genetic studies confirmed CHH together with IGF-1 deficiency. For this reason, we propose IGF-1 replacement therapy for its well-known actions on hematopoiesis, immune function and maturation, and metabolism. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Diminutive Porcelain Ascending Aorta With Supravalvular Aortic Stenosis.

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Houmsse, Mustafa; McDavid, Asia; Kilic, Ahmet

2018-05-01

This report describes the case of a 49-year-old man with a medical history significant for congenital aortic stenosis. The patient presented with progressive shortness of breath and decreased stamina and was found to have a concentric, diminutive porcelain ascending aorta with diffuse supravalvular aortic stenosis. We describe treatment with an aortic root augmentation and Bentall procedure using hypothermic circulatory arrest. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Prevalence of dental anomalies and enamel hypoplasia in primary dentition among preschool children of West Godavari District, Andhra Pradesh -A cross - sectional study

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Suzan Sahana

2013-01-01

Full Text Available Background: It is axiomatic that Pediatric dental anomalies and enamel hypoplasia (E.H are routinely encountered in primary dentition and early detection and prudent management of the condition facilitates normal occlusal development. Objectives: To determine the prevalence of various dental anomalies and enamel hypoplasia in preschool children between two to six years of age. Materials & Method: A total of 1898 children, between two to six years were randomly selected and screened for dental anomalies and enamel hypoplasia The chi square test was used to analyze the data statistically. Results: The overall prevalence rate of dental anomalies and enamel hypoplasia in this study was 0.63% and 8.95% respectively. Double teeth were the most frequently reported dental anomaly while supernumerary teeth were least reported. None of them reported with hypodontia.

Ascending Aortic Wall Cohesion: Comparison of Bicuspid and Tricuspid Valves

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Jaroslav Benedik

2012-01-01

Full Text Available Objectives. Bicuspid aortic valve (AV represents the most common form of congenital AV malformation, which is frequently associated with pathologies of the ascending aorta. We compared the mechanical properties of the aortic wall between patients with bicuspid and tricuspid AV using a new custom-made device mimicking transversal aortic wall shear stress. Methods. Between 03/2010 and 07/2011, 190 consecutive patients undergoing open aortic valve replacement at our institution were prospectively enrolled, presenting either with a bicuspid (group 1, n=44 or a tricuspid (group 2, n=146 AV. Aortic wall specimen were examined with the “dissectometer” resulting in nine specific aortic-wall parameters derived from tensile strength curves (TSC. Results. Patients with a bicuspid AV showed significantly more calcified valves (43.2% versus 15.8%, P<0.001, and a significantly thinner aortic wall (2.04±0.42 mm versus 2.24±0.41 mm, P=0.008. Transesophageal echocardiography diameters (annulus, aortic sinuses, and sinotubular junction were significantly larger in the bicuspid group (P=0.003, P=0.02, P=0.01. We found no difference in the aortic wall cohesion between both groups as revealed by shear stress testing (P=0.72, P=0.40, P=0.41. Conclusion. We observed no differences of TSC in patients presenting with tricuspid or bicuspid AVs. These results may allow us to assume that the morphology of the AV and the pathology of the ascending aorta are independent.

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

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

2017-07-01

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

Enamel hypoplasia in deciduous teeth of great apes: do differences in defect prevalence imply differential levels of physiological stress?

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Lukacs, J R

1999-11-01

This paper presents new data on enamel hypoplasia in the deciduous canine teeth of great apes. The enamel defect under consideration is known as localized hypoplasia of primary canines (LHPC), and is characterized by an area of thin or missing enamel on the labial surface of deciduous canine teeth (Skinner [1986a] Am. J. Phys. Anthropol. 69:59-69). Goals of this study are: 1) to determine if significant differences in the frequency of LHPC occur among three genera of great apes, and 2) to evaluate variation in LHPC prevalence among great apes as evidence of differential physiological stress. Infant and juvenile apes with deciduous teeth were examined at the Cleveland Museum of Natural History (n = 100) and at the Smithsonian Institution, National Museum of Natural History (n = 36). Deciduous teeth were observed under oblique incandescent light, with the naked eye and with a 10x hand lens. Enamel hypoplasia was scored using Federation Dentaire International (FDI)-Defects of Dental Enamel (DDE) standards. Hypoplasias were recorded by drawing defect location and size on a dental chart, and by measuring defect size and location with Helios needlepoint dial calipers. The prevalence of LHPC is reported by genus and sex, using two approaches: 1) the frequency of affected individuals-those having one or more deciduous canine teeth scored positive for LHPC; and 2) the number of canine teeth scored positive for LHPC as a percentage of all canine teeth observed. Variation in defect size and location will be described elsewhere. Localized hypoplasia of primary canine teeth was found in 62.5% of 128 individual apes, and in 45.5% of 398 great ape deciduous canines. As in humans, LHPC is the most common form of enamel hypoplasia in deciduous teeth of great apes, while LEH is rare or absent. The distribution and pattern of expression of LHPC in great apes is similar to that described in humans: side differences are not significant, but mandibular canines exhibit the defect two to

Aortic regurgitation after valve-sparing aortic root replacement: modes of failure.

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Oka, Takanori; Okita, Yutaka; Matsumori, Masamichi; Okada, Kenji; Minami, Hitoshi; Munakata, Hiroshi; Inoue, Takeshi; Tanaka, Akiko; Sakamoto, Toshihito; Omura, Atsushi; Nomura, Takuo

2011-11-01

Despite the positive clinical results of valve-sparing aortic root replacement, little is known about the causes of reoperations and the modes of failure. From October 1999 to June 2010, 101 patients underwent valve-sparing aortic root replacement using the David reimplantation technique. The definition of aortic root repair failure included the following: (1) intraoperative conversion to the Bentall procedure; (2) reoperation performed because of aortic regurgitation; and (3) aortic regurgitation equal to or greater than a moderate degree at the follow-up. Sixteen patients were considered to have repair failure. Three patients required intraoperative conversion to valve replacement, 3 required reoperation within 3 months, and another 8 required reoperation during postoperative follow-up. At initial surgery 5 patients had moderate to severe aortic regurgitation, 6 patients had acute aortic dissections, 3 had Marfan syndrome, 2 had status post Ross operations, 3 had bicuspid aortic valves, and 1 had aortitis. Five patients had undergone cusp repair, including Arantius plication in 3 and plication at the commissure in 2. The causes of early failure in 6 patients included cusp perforation (3), cusp prolapse (3), and severe hemolysis (1). The causes of late failure in 10 patients included cusp prolapse (4), commissure dehiscence (3), torn cusp (2), and cusp retraction (1). Patients had valve replacements at a mean of 23 ± 20.9 months after reimplantation and survived. Causes of early failure after valve-sparing root replacement included technical failure, cusp lesions, and steep learning curve. Late failure was caused by aortic root wall degeneration due to gelatin-resorcin-formalin glue, cusp degeneration, or progression of cusp prolapse. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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

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

1996-01-01

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

Transcatheter aortic valve replacement

Science.gov (United States)

... gov/ency/article/007684.htm Transcatheter aortic valve replacement To use the sharing features on this page, please enable JavaScript. Transcatheter aortic valve replacement (TAVR) is surgery to replace the aortic valve. ...

Percutaneous implantation of the first repositionable aortic valve prosthesis in a patient with severe aortic stenosis.

Science.gov (United States)

Buellesfeld, Lutz; Gerckens, Ulrich; Grube, Eberhard

2008-04-01

Percutaneous aortic valve replacement is a new less-invasive alternative for high-risk surgical candidates with aortic stenosis. However, the clinical experience is still limited, and the currently available 'first-generation devices' revealed technical shortcomings, such as lack of repositionability and presence of paravalvular leakages. We report the first-in-man experience with the new self-expanding Lotus Valve prosthesis composed of a nitinol frame with implemented bovine pericardial leaflets which is designed to address these issues, being repositionable and covered by a flexible membrane to seal paravalvular gaps. We implanted this prosthesis in a 93-year old patient presenting with severe symptomatic aortic stenosis (valve area: 0.6 cm(2)). Surgical valve replacement had been declined due to comorbidities. We used a retrograde approach for insertion of the 21-French Lotus catheter loaded with the valve prosthesis via surgical cut-down to the external iliac artery. Positioning of the valve was guided by transesophageal echo and supra-aortic angiograms. The prosthesis was successfully inserted and deployed within the calcified native valve. Echocardiography immediately after device deployment showed a significant reduction of the transaortic mean pressure gradient (32 to 9 mmHg; final valve area 1.7 cm(2)) without evidence of residual aortic regurgitation. The postprocedural clinical status improved from NYHA-IV to NYHA-II. These results remained unchanged up to the 3 month follow-up. Successful percutaneous aortic valve replacement can be performed using the new self-expanding and repositionable Lotus valve for treatment of high-risk patients with aortic valve stenosis. Further studies are mandatory to assess device safety and efficacy in larger patient populations. Copyright 2008 Wiley-Liss, Inc.

Ischio-pubic-patellar hypoplasia: is it a new syndrome?

International Nuclear Information System (INIS)

Habboub, H.K.; Thneibat, W.A.

1997-01-01

Aplasia/hypoplasia of the patella has been described as an isolated finding or, more commonly, as a part of congenital syndromes. We describe here bilateral absence of the patella in an 11-year-old girl with absence of the ischial and inferior pubic rami bilaterally. Other associated skeletal and soft-tissue deformities are also reported. To our knowledge, the constellation of these findings has not been described previously and represents a unique syndrome. (orig.). With 1 fig

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

International Nuclear Information System (INIS)

Ishizaka, Nobukazu; Sohmiya, Koichi; Miyamura, Masatoshi; Umeda, Tatsuya; Tsuji, Motomu; Katsumata, Takahiro; Miyata, Tetsuro

2012-01-01

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)

Aorto-aortic intrathoracic bypass in surgical treatment of aortic

International Nuclear Information System (INIS)

Gutierrez Perez, F.; Duran Reyes, A.; Bigalli, D.; Filgueira Berobide, J.

1998-01-01

The prevalence of coarctation of the aorta is 6.5 percent of all congenital heart defects, according to national and international data. There is a restenosis rate of patients after surgery. Factors that influence this evolution depends on the age at which patients underwent surgery for the first time the anatomy of the aortic arch and type of surgical technique. Several procedures can be used to correct the coarctation, which include surgery and balloon catheter dilation. We present here a case of a patient of 22 years old, with a recurrent coarctation of the aorta studied by echocardiography and magnetic resonance imaging. The patient underwent surgery a third time. We used an anterior approach (median sternotomy) and performed an aortic bypass graft, intrathoracic, under cardiopulmonary bypass. Evolved favorably and was discharged on the sixth day of post operative day (Author) [es

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

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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.

Efficiency of aortic valve commissurotomy for congenital aortic valve stenosis in pediatric population

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А. А. Лукьянов

2015-10-01

Full Text Available Background. This study was designed to evaluate short-term and long-term outcomes after open aortic valve commissurotomy in the pediatric patients at our center over a period of 10 years. Methods. A retrospective study of 94 patients who underwent open surgical commissurotomy because of aortic valve stenosis between 2003 and 2013 is presented. Follow-up time was in the range of 1 to 7 years. Results. The open aortic valve commissurotomy combined with debridement of leaflet free edge 36.1 %, LVOT myectomy 11.7%, leaflet suture plastic 7.4%, modified Konno procedure 6.3%, Brom's aortoplasty 4.2% was performed for all patients. Average cardiopulmonary bypass time was 59.2 30.7 min. Median aortic cross-clamping time was 31.52 15.1 min. Hospital complications were observed in 38.2% of cases. ICU time was in the range of 1 to 31 (mean 3.2 5.1 day. Artificial lung ventilation time varied from 2 to 76 (mean 15.3 18.3 hours. Inotropic support was needed in 26.5% of cases. Mean hospital stay time was 17.1 7.3 days. At follow-up between 2 and 7 years, reoperations were required for 3 patients who underwent a Ross procedure. Conclusions. The results evidence that open surgical commissurotomy is an effective way of treatment for aortic valve stenosis, considering the accuracy of plasty and additional techniques of leaflet correction. The best children age group for this type of operation is from first to five years of life.

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

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

2004-02-01

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

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

DEFF Research Database (Denmark)

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

2009-01-01

Transcatheter aortic valve implantation (TAVI) in severe aortic stenosis has proven to be a feasible and effective treatment modality for inoperable patients. Until now, neither aortic regurgitation nor degenerated bioprostheses has been an indication for TAVI. However, this article reports...... a successful valve-in-valve implantation of a CoreValve aortic valve prosthesis through the right subclavian artery in a case of severe aortic regurgitation within a degenerated aortic homograft. The case exemplifies the possibilities of expanding the indications for TAVI, as well as other vascular access...

Aortic dissection presenting with secondary pulmonary hypertension caused by compression of the pulmonary artery by dissecting hematoma: a case report

International Nuclear Information System (INIS)

Kim, Dong Hun; Ryu, Sang Wan; Choi, Yong Sun; Ahn, Byoung Hee

2004-01-01

The rupture of an acute dissection of the ascending aorta into the space surrounding the pulmonary artery is an uncommon occurrence. No previous cases of transient pulmonary hypertension caused by a hematoma surrounding the pulmonary artery have been documented in the literature. Herein, we report a case of acute aortic dissection presenting as secondary pulmonary hypertension

Aortocaval fistula – rare complication of ruptured abdominal aortic ...

African Journals Online (AJOL)

in 0.2 - 1.3% of patients with degenerative aortic aneurysms. 1 This paper describes the presentation and successful management of a patient with such an ACF. We wish to highlight the need to maintain a high index of suspicion for this condition in patients with abdominal aortic aneurysms (AAA), particularly if they present ...

Aortic valve-sparing operations in aortic root aneurysms: remodeling or reimplantation?

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Rahnavardi, Mohammad; Yan, Tristan D; Bannon, Paul G; Wilson, Michael K

2011-08-01

A best evidence topic was written according to a structured protocol. The question addressed was whether the reimplantation (David) technique or the remodeling (Yacoub) technique provides the optimum event free survival in patients with an aortic root aneurysm suitable for an aortic valve-sparing operation. In total, 392 papers were found using the reported search criteria, of which 14 papers provided the best evidence to answer the clinical question. A total of 1338 patients (Yacoub technique in 606 and David technique in 732) from 13 centres were included. In most series, cardiopulmonary bypass time and aortic cross-clamp time were longer for the David technique compared to the Yacoub technique. Early mortality was comparable between the two techniques (0-6.9% for the Yacoub technique and 0-6% for the David technique). There is a tendency for a higher freedom from significant long-term aortic insufficiency in the David group than the Yacoub group, which does not necessarily result in a higher reoperation rate in the Yacoub group. In the largest series reported, freedom from a moderate-to-severe aortic insufficiency at 12 years was 82.6 ± 6.2% in the Yacoub and 91.0 ± 3.8% in the David group (P=0.035). Freedom from reoperation at the same time point was 90.4 ± 4.7% in the Yacoub group and 97.4 ± 2.2% in the David group (P=0.09). In another series, freedom from reoperation at a follow-up time of about four years was 89 ± 4% in the Yacoub group and 98 ± 2% in the David group. Although some authors merely preferred the Yacoub technique for a bicuspid aortic valve, the accumulated evidence in the current review indicates comparable results for both techniques in a bicuspid aortic valve. Current evidence is in favour of the David rather than the Yacoub technique in pathologies such as Marfan syndrome, acute type A aortic dissection, and excessive annular dilatation that may impair aortic root integrity. Careful selection of patients for each technique and

Incidence and progression of mild aortic regurgitation after Tirone David reimplantation valve-sparing aortic root replacement.

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Stephens, Elizabeth H; Liang, David H; Kvitting, John-Peder Escobar; Kari, Fabian A; Fischbein, Michael P; Mitchell, R Scott; Miller, D Craig

2014-01-01

The study objective was to determine whether recurrent or residual mild aortic regurgitation, which occurs after valve-sparing aortic root replacement, progresses over time. Between 2003 and 2008, 154 patients underwent Tirone David-V valve-sparing aortic root replacement; 96 patients (62%) had both 1-year (median, 12 ± 4 months) and mid-term (62 ± 22 months) transthoracic echocardiograms available for analysis. Age of patients averaged 38 ± 13 years, 71% were male, 31% had a bicuspid aortic valve, 41% had Marfan syndrome, and 51% underwent aortic valve repair, predominantly cusp free margin shortening. Forty-one patients (43%) had mild aortic regurgitation on 1-year echocardiogram. In 85% of patients (n = 35), mild aortic regurgitation remained stable on the most recent echocardiogram (median, 57 ± 20 months); progression to moderate aortic regurgitation occurred in 5 patients (12%) at a median of 28 ± 18 months and remained stable thereafter; severe aortic regurgitation developed in 1 patient, eventually requiring reoperation. Five patients (5%) had moderate aortic regurgitation at 1 year, which did not progress subsequently. Two patients (2%) had more than moderate aortic regurgitation at 1 year, and both ultimately required reoperation. Although mild aortic regurgitation occurs frequently after valve-sparing aortic root replacement, it is unlikely to progress over the next 5 years and should not be interpreted as failure of the valve-preservation concept. Further, we suggest that mild aortic regurgitation should not be considered nonstructural valve dysfunction, as the 2008 valve reporting guidelines would indicate. We need 10- to 15-year follow-up to learn the long-term clinical consequences of mild aortic regurgitation early after valve-sparing aortic root replacement. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

An unusual case of aortic rupture after deployment of a bare stent in the treatment of aortic dissection in a patient with giant-cell arteritis.

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Rynio, Pawel; Kazimierczak, Arkadiusz; Gutowski, Piotr; Cnotliwy, Miloslaw

2017-06-01

Giant-cell arteritis is associated with a higher risk of aortic aneurysm and aortic dissection formation. We present a women with aortic dissection type B treated with a stent graft and bare-metal stent implantation. After the stent deployment we noticed aortic rupture, which was successfully treated with implantation of an additional stent graft. This report highlights the difficulty of endovascular therapy in patients with giant-cell arteritis. We have to bear in mind that chronic inflammation of the aorta leads to a more fragile aortic wall than normal. We recommend the use of a stent graft over a bare-metal stent and gentle use of a balloon catheter.

Indirect veneer treatment of anterior maxillary teeth with enamel hypoplasia

OpenAIRE

Juniarti, Devi Eka

2010-01-01

Background: Nowadays, aesthetic rehabilitation becomes a necessity. It is affected by patient’s background, especially career, social and economic status. The aesthetic abnormality of anterior teeth i.e discoloration, malposition and malformation can affect patient’s appearance, especially during smile. These dental abnormalities, as a result, can decrease patient’s performance. Dental malformation, for instance, can be caused by developmental tooth defect, such as enamel hypoplasia. Enamel h...

Total Endovascular Aortic Repair in a Patient with Marfan Syndrome.

Science.gov (United States)

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

2017-02-01

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

Comparing American, European and Asian practice guidelines for aortic diseases.

Science.gov (United States)

Rozado, Jose; Martin, Maria; Pascual, Isaac; Hernandez-Vaquero, Daniel; Moris, Cesar

2017-05-01

The aortic disease comprises a group of different pathologies of high prevalence, seriousness and ever changing by the medical and surgical investigations. Therefore cardiovascular scientific societies in USA, Europe and Asia have created Task Force on practice guidelines (PG) to develop, update and revise PG for aortic diseases. These documents issue recommendations on the diagnosis and management of different aortic diseases. The three societies agree on the recommendations about diagnostic tests and on the value of computed tomography and magnetic resonance as the main tools for the diagnosis and follow-up of aortic disease. Concerning to acute aortic syndromes (AAS), American and European GPs recognize intramural hematoma (IMH) as a type of AAS with surgery indication; however Asian guidelines consider IMH a pathological process different from AAS and indicate medical treatment. In thoracic aortic aneurysms (TAA), all express the need for an adequate control of cardiovascular risk factors, emphasizing strict control of blood pressure, smoking cessation and recommend the use of beta-blockers and statins. The threshold for asymptomatic repair is 5.5 cm in European and American and 6 cm for Asian PG, with lower thresholds in Marfan and bicuspid aortic valve (BAV). As regards the abdominal aortic aneurysms (AAA), the PGs recognize the adequate control of cardiovascular risk factors, but there are differences in class of recommendation on statins, angiotensin-converting enzyme inhibitors or beta-blockers to prevent progression of AAA. For intervention, the threshold diameter in asymptomatic is 5.5 cm but can be reduced to 5 cm in women as recommended by Asian PG. Moreover the specific diseases such as Marfan, BAV, pregnancy or atherosclerosis aortic present specific recommendations with small differences between PGs. In conclusion, PGs are interesting and appropriate documents at present. They issue recommendations based on evidence that help the clinician and

Reverse extra-anatomic aortic arch debranching procedure allowing thoracic endovascular aortic repair of a chronic ascending aortic aneurysm

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Ludovic Canaud, MD, PhD

2018-06-01

Full Text Available A 79-year-old woman was admitted with a large chronic dissecting ascending aortic aneurysm starting 5 mm distal to the ostia of the left coronary artery and ending immediately proximal to the innominate artery. A reverse extra-anatomic aortic arch debranching procedure was performed. During the same operative time, through a transapical approach, a thoracic stent graft was deployed with the proximal landing zone just distal to the coronary ostia and the distal landing zone excluding the origin of the left common carotid artery. The postoperative course was uneventful. Computed tomography at 12 months documented patent extra-anatomic aortic arch debranching and no evidence of endoleak. Keywords: Ascending aorta, Thoracic aorta, Aortic dissection, Stent graft

Retrograde ascending aortic dissection during or after thoracic aortic stent graft placement: insight from the European registry on endovascular aortic repair complications

DEFF Research Database (Denmark)

Eggebrecht, Holger; Thompson, Matt; Rousseau, Hervé

2009-01-01

BACKGROUND: Single-center reports have identified retrograde ascending aortic dissection (rAAD) as a potentially lethal complication of thoracic endovascular aortic repair (TEVAR). METHODS AND RESULTS: Between 1995 and 2008, 28 centers participating in the European Registry on Endovascular Aortic...

Hybrid treatment of aortic arch disease

Science.gov (United States)

Metzger, Patrick Bastos; Rossi, Fabio Henrique; Moreira, Samuel Martins; Issa, Mario; Izukawa, Nilo Mitsuru; Dinkhuysen, Jarbas J.; Spina Neto, Domingos; Kambara, Antônio Massamitsu

2014-01-01

Introduction The management of thoracic aortic disease involving the ascending aorta, aortic arch and descending thoracic aorta are technically challenging and is an area in constant development and innovation. Objective To analyze early and midterm results of hybrid treatment of arch aortic disease. Methods Retrospective study of procedures performed from January 2010 to December 2012. The end points were the technical success, therapeutic success, morbidity and mortality, neurologic outcomes, the rate of endoleaks and reinterventions. Results A total of 95 patients treated for thoracic aortic diseases in this period, 18 underwent hybrid treatment and entered in this study. The average ages were 62.3 years. The male was present in 66.7%. The technical and therapeutic success was 94.5% e 83.3%. The perioperative mortality rate of 11.1%. There is any death during one-year follow- up. The reoperation rates were 16.6% due 2 cases of endoleak Ia and one case of endoleak II. There is any occlusion of anatomic or extra anatomic bypass during follow up. Conclusion In our study, the hybrid treatment of aortic arch disease proved to be a feasible alternative of conventional surgery. The therapeutic success rates and re- interventions obtained demonstrate the necessity of thorough clinical follow-up of these patients in a long time. PMID:25714205

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

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Costa Mario Gesteira

2002-01-01

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

Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases

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V Govindaraj

2017-01-01

Full Text Available Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

Single-center experience using the Freedom SOLO aortic bioprosthesis.

Science.gov (United States)

Iliopoulos, Dimitrios C; Deveja, Aris Rezar; Androutsopoulou, Vasiliki; Filias, Vasilios; Kastelanos, Eleftherios; Satratzemis, Vasilios; Khalpey, Zain; Koudoumas, Dimitrios

2013-07-01

This study reviews a single institution experience with the Freedom SOLO (Sorin Group, Saluggia, Italy) aortic bioprosthesis. Between October 2006 and February 2010, 128 patients (64 men, 64 women; mean age, 75.8 ± 5.1 years) underwent aortic valve replacement using the Freedom SOLO stentless aortic valve. The follow-up time was 36.7 ± 1.2 months and 100% complete. Concomitant procedures were performed in 77 patients (60%). The mean standard European System for Cardiac Operative Risk Evaluation was 9 ± 2.7. Grade 3 aortic stenosis was present in 73% of patients, mixed aortic stenosis and regurgitation were present in 40% of patients, and mitral regurgitation was present in 46% of patients. The mean crossclamp time was 53 ± 12 minutes for isolated Freedom SOLO aortic valve implantation and 80 ± 28 minutes for concomitant procedures, and the mean cardiopulmonary bypass time was 103 ± 31 minutes. The mean implanted valve size was 22.6 ± 1.4 mm. The mean intensive care unit and hospital stays were 2.4 ± 1.1 days and 8.8 ± 2.6 days, respectively. Three patients underwent reoperation for bleeding. The 15-day, 30-day, and perioperative mortality were all 4.6%. The 36-month survival was 95.4% ± 1.6% for the cohort with a low European System for Cardiac Operative Risk Evaluation (9). Echocardiographic data preoperatively, immediately postoperatively, and at 3, 6, and 12 months postoperatively showed peak transvalvular gradients of 75 ± 23, 17 ± 6, 18 ± 6.5, 16 ± 6, and 16 ± 9 mm Hg, respectively (P SOLO stentless aortic valve has excellent early and intermediate-term results. Published by Mosby, Inc.

Neurotrophin 3 upregulates proliferation and collagen production in human aortic valve interstitial cells: a potential role in aortic valve sclerosis.

Science.gov (United States)

Yao, Qingzhou; Song, Rui; Ao, Lihua; Cleveland, Joseph C; Fullerton, David A; Meng, Xianzhong

2017-06-01

Calcific aortic valve disease (CAVD) is a leading cardiovascular disorder in the elderly. Diseased aortic valves are characterized by sclerosis (fibrosis) and nodular calcification. Sclerosis, an early pathological change, is caused by aortic valve interstitial cell (AVIC) proliferation and overproduction of extracellular matrix (ECM) proteins. However, the mechanism of aortic valve sclerosis remains unclear. Recently, we observed that diseased human aortic valves overexpress growth factor neurotrophin 3 (NT3). In the present study, we tested the hypothesis that NT3 is a profibrogenic factor to human AVICs. AVICs isolated from normal human aortic valves were cultured in M199 growth medium and treated with recombinant human NT3 (0.10 µg/ml). An exposure to NT3 induced AVIC proliferation, upregulated the production of collagen and matrix metalloproteinase (MMP), and augmented collagen deposition. These changes were abolished by inhibition of the Trk receptors. NT3 induced Akt phosphorylation and increased cyclin D1 protein levels in a Trk receptor-dependent fashion. Inhibition of Akt abrogated the effect of NT3 on cyclin D1 production. Furthermore, inhibition of either Akt or cyclin D1 suppressed NT3-induced cellular proliferation and MMP-9 and collagen production, as well as collagen deposition. Thus, NT3 upregulates cellular proliferation, ECM protein production, and collagen deposition in human AVICs. It exerts these effects through the Trk-Akt-cyclin D1 cascade. NT3 is a profibrogenic mediator in human aortic valve, and overproduction of NT3 by aortic valve tissue may contribute to the mechanism of valvular sclerosis. Copyright © 2017 the American Physiological Society.

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

Science.gov (United States)

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

2018-04-01

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

Aortic obstruction: anatomy and echocardiography

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Keirns Candace

2006-09-01

Full Text Available Abstract Echocardiography is a valuable non-invasive technique for identifying the site and type of aortic obstruction. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. This study was undertaken to correlate the echocardiographic images with anatomic specimens of equivalent valvular and supravalvular aortic obstruction. Specimens were part of the collection of the Department of Embryology. Fifty six patients were studied, and forty specimens with aortic obstruction were analyzed. Echocardiographic characteristics: Thirty one (55.3% patients were women and twenty five (44.7% men. Valvular aortic obstruction was found in Thirty six patients (64.3 % and supravalvular aortic obstruction in twenty (35.7%. Anatomic characteristics: Of the forty specimens examined, twenty one (52.5% had valvular aortic obstruction and nineteen (47.5% supravalvular aortic obstruction. The anatomoechocardiographic correlation clearly showed that the anatomic findings of the specimen hearts and aortas corresponded to echocardiographic images of valvular and supravalvular aortic obstruction and provided solid corroboration of echocardiographic diagnoses.

Unusual Case of Overt Aortic Dissection Mimicking Aortic Intramural Hematoma

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Kushtrim Disha

2016-04-01

Full Text Available 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.

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

Science.gov (United States)

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

2017-07-01

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

Brain strokes related to aortic aneurysma – the analysis of three cases

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Pastuszak Żanna

2017-04-01

Full Text Available Brain stroke connected with aortic blood flow disturbances is a rare disease and its incidence is difficult to assume. Nevertheless, 10-50% of patients with aortic dissection may not experience any pain. In case of 18-30% patients with aortic dissection neurological signs are first disease presentation and among them ischemic stroke is the most common. The most popular aortic dissection classification is with use of Stanford system. Type A involves the ascending aorta and type B is occurring distal to the subclavian artery. Aortic dissection risk factors include hypertension, cystic medionecrosis, bicuspid aortic valve and Marfan’s or Ehlers-Danlos syndrome.

Sequential transcatheter aortic valve implantation due to valve dislodgement

DEFF Research Database (Denmark)

Campante Teles, Rui; Costa, Cátia; Almeida, Manuel

2017-01-01

Transcatheter aortic valve implantation (TAVI) has become an important treatment in high surgical risk patients with severe aortic stenosis (AS), whose complications need to be managed promptly. The authors report the case of an 86-year-old woman presenting with severe symptomatic AS, rejected...

Survival and freedom from aortic valve-related reoperation after valve-sparing aortic root replacement in 1015 patients.

Science.gov (United States)

Kari, Fabian A; Doll, Kai-Nicolas; Hemmer, Wolfgang; Liebrich, Markus; Sievers, Hans-Hinrich; Richardt, Doreen; Reichenspurner, Hermann; Detter, Christian; Siepe, Matthias; Czerny, Martin; Beyersdorf, Friedhelm

2016-04-01

The aim of this study was to characterize mortality and aortic valve replacement after valve-sparing aortic root replacement (V-SARR) in a multicentre cohort. Between 1994 and 2014, 1015 patients had V-SARR with (n = 288, 28%) or without cusp/commissure repair (n = 727, 72%) at the centres of Lübeck (n = 343, 34%), Stuttgart (n = 346, 34%), Hamburg (n = 109, 11%) and Freiburg (n = 217, 21%), Germany. Comparative survival of an age- and gender-matched general population was calculated. Log-rank tests and multiple logistic regression were used to identify risk factors. The mean follow-up was 5.2 ± 3.9 years. Cumulative follow-up comprised 2933 patient-years. Early survival was 98%. NYHA status and aneurysm size were predictive of death during mid-term follow-up (P = 0.025). Freedom from aortic valve replacement was 90% at 8 years, with the type of V-SARR (root remodelling, David II) being a risk factor (P = 0.015). Bicuspid aortic valve (P = 0.26) and initial valve function (P = 0.4) did not impact reoperation. The need of additional valve repair (cusps/commissures) was not linked to reoperation: freedom from aortic valve replacement at 8 years was 84% if cusp repair was performed versus 90% if V-SARR alone was performed (P = 0.218). Marfan syndrome had no impact on survival or on aortic valve replacement. Mid-term survival of patients after V-SARR is comparable with that of a matched general population. The regurgitant bicuspid aortic valve is a favourable substrate for V-SARR. Prophylactic surgery should be performed before symptoms or large aneurysms are present to achieve optimal mid-term outcomes. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

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

International Nuclear Information System (INIS)

Zhi Aihua; Dai Ruping; Jiang Shiliang; Lu Bin

2012-01-01

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)

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

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Knollmann, Friedrich D., E-mail: friedrich.knollmann@ucdmc.ucdavis.edu [Department of Radiology, University of California, Davis, 4860 Y Street, Sacramento, CA 95817 (United States); Departments of Radiology and Cardiothoracic Surgery, University of Pittsburgh, 200 Lothrop Street, Pittsburgh, PA 15213 (United States); Lacomis, Joan M.; Ocak, Iclal; Gleason, Thomas [Department of Radiology, University of California, Davis, 4860 Y Street, Sacramento, CA 95817 (United States); Departments of Radiology and Cardiothoracic Surgery, University of Pittsburgh, 200 Lothrop Street, Pittsburgh, PA 15213 (United States)

2013-12-01

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.

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

International Nuclear Information System (INIS)

Knollmann, Friedrich D.; Lacomis, Joan M.; Ocak, Iclal; Gleason, Thomas

2013-01-01

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

A practical tip to engage the left main coronary artery in patients presenting with aortic aneurysms

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Kudret Keskin

2017-09-01

Full Text Available Coronary angiography in patients presenting with ascending aortic aneurysms can be challenging since standard catheters may fail to reach the left main coronary artery. A widely accepted approach is the mother-in-child catheter technique whereby a catheter with a smaller diameter is telescoped through one with a bigger diameter, a procedure that helps to lengthen the catheter. However, this technique is associated with several disadvantages such as thrombus formation within the catheters, inadvertent damage to the left main ostium, and the necessity of manipulation, which the operator may not be familiar with. Therefore, in our case we present a method, which involves the application of a regular Judkins Right 4 catheter for left main engagement. We propose that this method can be safely attempted prior to using telescoping techniques.

3D echocardiographic analysis of aortic annulus for transcatheter aortic valve replacement using novel aortic valve quantification software: Comparison with computed tomography.

Science.gov (United States)

Mediratta, Anuj; Addetia, Karima; Medvedofsky, Diego; Schneider, Robert J; Kruse, Eric; Shah, Atman P; Nathan, Sandeep; Paul, Jonathan D; Blair, John E; Ota, Takeyoshi; Balkhy, Husam H; Patel, Amit R; Mor-Avi, Victor; Lang, Roberto M

2017-05-01

With the increasing use of transcatheter aortic valve replacement (TAVR) in patients with aortic stenosis (AS), computed tomography (CT) remains the standard for annulus sizing. However, 3D transesophageal echocardiography (TEE) has been an alternative in patients with contraindications to CT. We sought to (1) test the feasibility, accuracy, and reproducibility of prototype 3DTEE analysis software (Philips) for aortic annular measurements and (2) compare the new approach to the existing echocardiographic techniques. We prospectively studied 52 patients who underwent gated contrast CT, procedural 3DTEE, and TAVR. 3DTEE images were analyzed using novel semi-automated software designed for 3D measurements of the aortic root, which uses multiplanar reconstruction, similar to CT analysis. Aortic annulus measurements included area, perimeter, and diameter calculations from these measurements. The results were compared to CT-derived values. Additionally, 3D echocardiographic measurements (3D planimetry and mitral valve analysis software adapted for the aortic valve) were also compared to the CT reference values. 3DTEE image quality was sufficient in 90% of patients for aortic annulus measurements using the new software, which were in good agreement with CT (r-values: .89-.91) and small (software can accurately measure aortic annulus in patients with severe AS undergoing TAVR, in better agreement with CT than the existing methodology. Accordingly, intra-procedural TEE could potentially replace CT in patients where CT carries significant risk. © 2017, Wiley Periodicals, Inc.

Dynamics of the aortic annulus in 4D CT angiography for transcatheter aortic valve implantation patients

NARCIS (Netherlands)

Elattar, Mustafa A.; Vink, Leon W.; van Mourik, Martijn S.; Baan, Jan; Vanbavel, Ed T.; Planken, R. Nils; Marquering, Henk A.

2017-01-01

Transcatheter aortic valve implantation (TAVI) is a well-established treatment for patients with severe aortic valve stenosis. This procedure requires pre-operative planning by assessment of aortic dimensions on CT Angiography (CTA). It is well-known that the aortic root dimensions vary over the

Pathogenetic Basis of Aortopathy and Aortic Valve Disease

Science.gov (United States)

2018-02-19

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

A detailed study of enamel hypoplasia in a post-medieval adolescent of known age and sex.

Science.gov (United States)

King, T; Hillson, S; Humphrey, L T

2002-01-01

Developmental disturbances that affect the secretion of enamel matrix can cause defective enamel structure. Linear hypoplasia is one type of enamel defect and manifests itself as a furrow that runs around the circumference of the tooth. Such defects range in size from the microscopic to those that are several millimetres wide. Enamel defects have been widely used by anthropologists for the investigation of growth disruptions in past populations, as they provide a permanent record of disturbances during much of a child's developmental period. This is a detailed case study of enamel growth disruptions in a 15-year-old female from the 18th and 19th century crypt of Christ Church, Spitalfields. The method used relates linear enamel hypoplasia to the incremental structures in the enamel surface, the perikymata, in order to investigate the timing of growth disturbances. Linear enamel hypoplasia was defined here as a greater than expected spacing between neighbouring pairs of perikymata. In addition, this study used recently published histological data on the precise timing of tooth development to establish chronologies for growth disruptions. Defects were matched in at least two teeth with overlapping developmental schedules to ensure that systemic disturbances, as opposed to localised traumas, were identified. Thirteen enamel defects were matched between five different teeth from the same individual from Spitalfields. Most linear enamel hypoplasias were evident on the anterior dentition. Using an 8-day average perikymata periodicity, the age at first defect in this individual was calculated as 1.5 years and the last growth disruption occurred when she was 4.6 years of age. The distribution of the defects was examined to identify any seasonal pattern in the occurrence of the growth disturbances.

Aortic valvuloplasty of calcific aortic stenosis with monofoil and trefoil balloon catheters: practical considerations

NARCIS (Netherlands)

S. Plante (Sylvain); M.J.B.M. van den Brand (Marcel); L.C.P. van Veen; C. di Mario (Carlo); C.E. Essed; K.J. Beatt (Kevin); P.W.J.C. Serruys (Patrick)

1990-01-01

textabstractIn order to evaluate the relation between balloon design (monofoil, trefoil) and valvular configuration, experimental aortic valvuloplasty was performed in four post-mortem hearts with calcific aortic stenosis of various morphology. The degree of obstruction of the aortic orifice was

Thoracic Endovascular Aortic Repair (TEVAR) in Proximal (Type A) Aortic Dissection: Ready for a Broader Application?

OpenAIRE

Nienaber, Christoph A.; Sakalihasan, Natzi; Clough, Rachel E.; Aboukoura, Mohamed; Mancuso, Enrico; Yeh, James S.M.; Defraigne, Jean-Olivier; Cheshire, Nick; Rosendahl, Ulrich Peter; Quarto, Cesare; Pepper, John

2016-01-01

ObjectiveThoracic endovascular aortic repair (TEVAR) has demonstrated encouraging results and is gaining increasing acceptance as a treatment option for aortic aneurysms and dissections. Yet, its role in managing proximal aortic pathologies is unknown - this is important because in proximal (Stanford type A) aortic dissections, 10-30% are not accepted for surgery, and 30-50% are technically amenable for TEVAR. We describe our case series of type A aortic dissections treated using TEVAR.Method...

Thrombosed false channel in acute aortic dissections

International Nuclear Information System (INIS)

Alerci, M.; Dore, R.; Kluzer, A.; Digiulio, G.; D'Andrea, F.; De Agostini, A.

1987-01-01

Three cases are reported of acute dissection with complete thrombosis of the false channel, which is a very uncommonevent in DeBakey's I and II type aortic dissections. The 3 patients entered hospital because of severe thoracic pain without any ECG sign of myocardial infarction. Emergency CT showed evidence of pericardial effusion suggesting hemopericardium, enlargement of the ascending aorta and a peripheral semilunar filling defect which caused a slight deformation of the true channel. On precontrast scans, only one case showed inward displacement of peripheral calcifications and high-density aortic wall. No tipical signs of aortic dissection were reported, expect in the first patient - where a double contrast filled lumen, separated by an intimal flap was seen. CT findings are individually discussed. It is emphasized that in all patients more than one CT sign was present at a time. The correct evaluation of these signs together with the clinacal data could lead to the right diagnosis of aortic dissection in spite of the lack of a f???

Unusual right ventricle aneurysm and dysplastic pulmonary valve with mitral valve hypoplasia

Directory of Open Access Journals (Sweden)

Ozge Pamukcu

2013-01-01

Full Text Available We report a newborn with an unusual combination of aneurysmally dilated thin-walled right ventricle with hypertrophy of the apical muscles of the right ventricle. There was narrow pulmonary annulus, pulmonary regurgitation, and hypoplasia of the mitral valve and left ventricle. We propose that this heart represents a partial form of Uhl`s anomaly.

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

Science.gov (United States)

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

2014-01-01

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

Postoperative Reverse Remodeling and Symptomatic Improvement in Normal-Flow Low-Gradient Aortic Stenosis After Aortic Valve Replacement.

Science.gov (United States)

Carter-Storch, Rasmus; Møller, Jacob E; Christensen, Nicolaj L; Irmukhadenov, Akhmadjon; Rasmussen, Lars M; Pecini, Redi; Øvrehus, Kristian A; Søndergård, Eva V; Marcussen, Niels; Dahl, Jordi S

2017-12-01

Severe aortic stenosis (AS) most often presents with reduced aortic valve area (gradient (≥40 mm Hg; normal-flow high-gradient AS) or low mean gradient (normal-flow low-gradient [NFLG] AS). The benefit of aortic valve replacement (AVR) among NFLG patients is controversial. We compared the impact of NFLG condition on preoperative left ventricular (LV) remodeling and myocardial fibrosis and postoperative remodeling and symptomatic benefit. Eighty-seven consecutive patients with reduced aortic valve area and normal stroke volume index undergoing AVR underwent echocardiography, magnetic resonance imaging, a 6-minute walk test, and measurement of natriuretic peptides before and 1 year after AVR. Myocardial fibrosis was assessed from magnetic resonance imaging. Patients were stratified as NFLG or normal-flow high-gradient. In total, 33 patients (38%) had NFLG. Before AVR, they were characterized by similar symptom burden but less severe AS measured by aortic valve area index (0.50±0.09 versus 0.40±0.08 cm 2 /m 2 ; P gradient condition independently predicted change in LV mass index. Patients with NFLG had less severe AS and LV remodeling than patients with normal-flow high-gradient. Furthermore, NFLG patients experienced less reverse remodeling but the same symptomatic benefit. URL: http://www.clinicaltrials.gov. Unique identifier: NCT02316587. © 2017 American Heart Association, Inc.

Consumption coagulopathy in acute aortic dissection: principles of management.

Science.gov (United States)

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

2017-06-12

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

Microarray analysis to identify the similarities and differences of pathogenesis between aortic occlusive disease and abdominal aortic aneurysm.

Science.gov (United States)

Wang, Guofu; Bi, Lechang; Wang, Gaofeng; Huang, Feilai; Lu, Mingjing; Zhu, Kai

2018-06-01

Objectives Expression profile of GSE57691 was analyzed to identify the similarities and differences between aortic occlusive disease and abdominal aortic aneurysm. Methods The expression profile of GSE57691 was downloaded from Gene Expression Omnibus database, including 20 small abdominal aortic aneurysm samples, 29 large abdominal aortic aneurysm samples, 9 aortic occlusive disease samples, and 10 control samples. Using the limma package in R, the differentially expressed genes were screened. Followed by enrichment analysis was performed for the differentially expressed genes using database for annotation, visualization, and integrated discovery online tool. Based on string online tool and Cytoscape software, protein-protein interaction network and module analyses were carried out. Moreover, integrated TF platform database and Cytoscape software were used for constructing transcriptional regulatory networks. Results As a result, 1757, 354, and 396 differentially expressed genes separately were identified in aortic occlusive disease, large abdominal aortic aneurysm, and small abdominal aortic aneurysm samples. UBB was significantly enriched in proteolysis related pathways with a high degree in three groups. SPARCL1 was another gene shared by these groups and regulated by NFIA, which had a high degree in transcriptional regulatory network. ACTB, a significant upregulated gene in abdominal aortic aneurysm samples, could be regulated by CLIC4, which was significantly enriched in cell motions. ACLY and NFIB were separately identified in aortic occlusive disease and small abdominal aortic aneurysm samples, and separately enriched in lipid metabolism and negative regulation of cell proliferation. Conclusions The downregulated UBB, NFIA, and SPARCL1 might play key roles in both aortic occlusive disease and abdominal aortic aneurysm, while the upregulated ACTB might only involve in abdominal aortic aneurysm. ACLY and NFIB were specifically involved in aortic occlusive

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

Bovine aortic arch with supravalvular aortic stenosis

Directory of Open Access Journals (Sweden)

Mohammed Idhrees

2016-09-01

Full Text Available A 5-year-old boy was diagnosed to have supravalvular aortic stenosis (SVAS. On evaluation of CT angiogram, there was associated bovine aortic arch (BAA. Association of BAA with SVAS has not been previously reported in literature, and to best of our knowledge, this is the first case report of SVAS with BAA. Recent studies show BAA as a marker for aortopathy. SVAS is also an arteriopathy. In light of this, SVAS can also possibly be a manifestation of aortopathy associated with BAA.

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

DEFF Research Database (Denmark)

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

2014-01-01

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

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

NARCIS (Netherlands)

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

2004-01-01

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

Current indications for stentless aortic bioprostheses.

Science.gov (United States)

Hegazy, Yasser Y; Rayan, Amr; Bauer, Stefan; Keshk, Noha; Bauer, Kerstin; Ennker, Ina; Ennker, Jürgen

2018-01-01

The best aortic prostheses have been debated for decades. The introduction of stentless aortic bioprostheses was aimed at improving hemodynamics and potentially the durability of aortic bioprostheses. Despite the good short- and long-term outcomes after implantation of stentless aortic bioprostheses, their use remains limited owing to the technically demanding implantation techniques. Nevertheless, stentless aortic bioprostheses might be of special benefit in certain indications, where they could be a valuable addition to the surgical armamentarium.

Aortopathy in Congenital Heart Disease in Adults: Aortic Dilatation with Decreased Aortic Elasticity that Impacts Negatively on Left Ventricular Function.

Science.gov (United States)

Niwa, Koichiro

2013-04-01

Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, tetralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessitates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: "aortopathy".

Minimally invasive aortic valve replacement

DEFF Research Database (Denmark)

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

2009-01-01

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

Transcatheter Aortic Valve Implantation in Lower-Risk Patients With Aortic Stenosis: Is It Justified to Be the Preferred Treatment?

Science.gov (United States)

Abdelghani, Mohammad; Serruys, Patrick W

2016-04-01

Transcatheter aortic valve implantation underwent progressive improvements until it became the default therapy for inoperable patients, and a recommended therapy in high-risk operable patients with symptomatic severe aortic stenosis. In the lower-risk patient strata, a currently costly therapy that still has important complications with questionable durability is competing with the established effective and still-improving surgical replacement. This report tries to weigh the clinical evidence, the recent technical improvements, the durability, and the cost-effectiveness claims supporting the adoption of transcatheter aortic valve implantation in intermediate-low risk patients. The importance of appropriate patients' risk stratification and a more comprehensive approach to estimate that risk are also emphasized in the present report. © 2016 American Heart Association, Inc.

A correlative study of aortic valve rotation angle and thoracic aortic sizes using ECG gated CT angiography

Energy Technology Data Exchange (ETDEWEB)

Saremi, Farhood, E-mail: fsaremi@usc.edu; Cen, Steven; Tayari, Nazila; Alizadeh, Houman; Emami, Amir; Lin, Leah; Fleischman, Fernando

2017-04-15

Objective: Various degrees of aortic valve rotation may be seen in individuals with no history of congenital cardiovascular malformations, but its association with aortic sizes has not been studied. Methods: Gated computed tomographic (CT angiograms in 217 patients were studied (66.7 ± 15; 22–97 years old)). Aortic diameters were determined at 5 anatomic locations. The length of the aorta from sinus to left subclavian artery was measured. The angle of valve rotation was recorded by measuring the angle between a line connecting the midpoint of the non-coronary sinus to the anterior commissure and another line along the interatrial septum. Rotation angles were correlated with aortic measurements. Patients were separated into two groups based on aortic sizes and into three groups based on age. The threshold for aortic dilatation was set at maximum ascending aorta diameter ≥40 mm (≥21 mm body surface area [BSA] indexed). Results: No significant difference in rotation angles was seen between the three age groups or between genders. Rotation angles were significantly correlated with maximal, average, and BSA adjustment of the aortic root and ascending aortic measurements. The aortic root angles were significantly different between the dilated versus nondilated aortas. There was no significant association between the rotation angles and age, length of ascending aorta, or diameters of descending aorta. Multivariate adaptive regression splines showed 25° of aortic root rotation as the diagnostic cut off for ascending aorta dilation. Above the 25° rotation, every 10° of increasing rotation was associated with a 3.78 ± 0.87 mm increase in aortic diameter (p < 0.01) and a 1.73 ± 0.25 times increased risk for having a dilated aorta (p < 0.01). Conclusion: Rotation angles of the aortic valve may be an independent non-invasive imaging marker for dilatation of the ascending aorta. Patients with increased rotation angle of the aortic valve may have higher risk for

Aortic intracardiac echocardiography-guided septal puncture during mitral valvuloplasty.

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Akkaya, Emre; Vuruskan, Ertan; Zorlu, Ali; Sincer, Isa; Kucukosmanoglu, Mehmet; Ardic, Idris; Yilmaz, Mehmet Birhan

2014-01-01

Transoesophageal echocardiography (TEE) and venous intracardiac echocardiography (ICE) are traditionally used to visualize the interatrial septum (IAS) and the tenting effect of the fossa ovalis in patients undergoing percutaneous balloon mitral valvuloplasty (PBMV). The aim of the present study was to assess the comparative efficacy and safety of arterial (intra-aortic) ICE and venous ICE, compared with TEE (traditional approach), in the patients undergoing PBMV. TEE, aortic ICE, and venous ICE were consecutively performed in 50 patients (40 ± 9 years, 86% female). The images of intracardiac structures were obtained from both aortic and right atrial loci. The IAS was visualized using TEE, aortic ICE, and venous ICE. The mean mitral valve area was 1.14 ± 0.2 cm(2), and the mean left atrial volume index was 57.5 ± 12 mL/m(2). The mean size of the visualized septal length was 48 ± 5 mm by TEE, 51 ± 5 mm by aortic ICE, and 33 ± 6 mm by venous ICE. The Bland-Altman test indicated that the 95% limits of agreement for the measurement of septal diameter ranged from -11.0 to +5.9 mm (mean -2.5 mm) between TEE and aortic ICE, -2.8 to +33.5 mm (mean +15.3 mm) between TEE and venous ICE, and -36.6 to +0.8 mm (mean -17.9 mm) between venous and aortic ICE. Standard venous ICE generally tended to yield smaller values compared with TEE and aortic ICE for the measurement of septal length. Furthermore, the view of fossa ovalis and 'tenting effect' was optimal in 11 patients on venous ICE; however, the fossa ovalis and tip of the needle were well visualized in all patients on aortic ICE (P < 0.001). There were no major complications with the use of aortic ICE. Aortic ICE is a superior alternative to venous ICE and facilitates trans-septal puncture in patients with mitral stenosis.

Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

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Webb, Emma A; O'Reilly, Michelle A; Clayden, Jonathan D; Seunarine, Kiran K; Dale, Naomi; Salt, Alison; Clark, Chris A; Dattani, Mehul T

2013-01-01

To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years) underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years) underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex) was performed. Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (pchildren with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, pchildren with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic radiations of children with mild to moderate or no visual impairment raises questions as to the pathogenesis of these changes which will need to be addressed by future studies.

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

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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.)

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

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

2015-11-01

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

Imaging techniques in transcatheter aortic valve replacement

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Quaife RA

2013-11-01

Full Text Available Robert A Quaife, Jennifer Dorosz, John C Messenger, Ernesto E Salcedo Division of Cardiology, University of Colorado, Aurora, CO, USA Abstract: Calcific aortic stenosis is now understood as a complex valvular degenerative process sharing many risk factors with atherosclerosis. Once patients develop symptomatic calcific aortic stenosis, the only effective treatment is aortic valve replacement. In the past decade, transcatheter aortic valve replacement (TAVR has been developed as an alternative to surgery to treat severe calcific aortic stenosis. Cardiac imaging plays a pivotal role in the contemporary management of patients with calcific aortic stenosis, and particularly in patients being considered for TAVR, who demand detailed imaging of the aortic valve apparatus. In this review, we highlight the role of cardiac imaging for patient selection, procedural guidance, and evaluation of results of TAVR. Keywords: aortic stenosis, cardiovascular imaging, transcutaneous aortic valve replacement

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

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

2015-02-01

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

The changing course of aortic valve disease in Scotland: temporal trends in hospitalizations and mortality and prognostic importance of aortic stenosis.

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Berry, Colin; Lloyd, Suzanne M; Wang, Yanzhong; Macdonald, Alyson; Ford, Ian

2013-06-01

To investigate the contemporary clinical course of aortic valve disease types. We performed a retrospective population-level epidemiological study of hospitalized care in Scotland from 1 January 1997 to 31 December 2005 using electronic case identification of hospital admissions and deaths. Time-to-event analyses were performed using Cox Proportional-Hazards models. A total of 19 733 adults with an index hospitalization and a final diagnosis of non-congenital aortic valve disease were identified. Aortic stenosis, aortic insufficiency, mixed aortic valve disease, or unspecified aortic valve disease occurred in 13 220 (67.0%), 2807 (14.2%), 699 (3.5%), and 3007 (15.2%), individuals, respectively. The majority of hospitalizations occurred in elderly persons aged 80 and older. In total, 9981 (50.6%) patients had died by 31 December 2006. When compared with aortic stenosis, the risk of death was less with aortic insufficiency [hazard ratio (95% confidence interval) 0.79 (0.74, 0.84)] and mixed aortic valve disease [0.83 (0.74, 0.93)]. Female gender, admission year, and hypertension were associated with lower mortality in patients with aortic stenosis. Patients with aortic stenosis had increased risk of death or heart failure (adjusted P valve replacement of whom 73.2% had aortic stenosis, 11.9% aortic valve disease (unspecified),10.0% aortic insufficiency, and 4.9% aortic stenosis with insufficiency. Patients with aortic stenosis with insufficiency had increased likelihood of aortic valve replacement [1.19 (1.02, 1.38)]. Age, female gender, and co-morbidity reduced the likelihood of aortic valve replacement. The incidence of aortic valve stenosis has substantially increased in Scotland in recent years. Aortic stenosis predicts morbidity and mortality when compared with other types of aortic valve disease.

Quantification of abdominal aortic deformation after EVAR

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

2009-02-01

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

Multiple subfailures characterize blunt aortic injury.

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Stemper, Brian D; Yoganandan, Narayan; Pintar, Frank A; Brasel, Karen J

2007-05-01

Blunt aortic injuries result from rapid deceleration of the thorax as may occur during automotive impacts and falls from extreme heights. Pathological findings can range from failure of specific vessel layers to immediate vessel wall rupture. The purpose of this investigation was to determine the sequence of local structural events that may lead to aortic wall disruption. Fourteen porcine aorta specimens were opened to expose the intima and longitudinally distracted until rupture. Longitudinal mechanics were quantified and subfailures were identified. Histology was used to examine internal layer subfailure. Videography demonstrated that subfailures propagated into complete vessel wall rupture. Subfailures occurred before complete vessel rupture in 93% of specimens. Intimal and medial subfailures were present at 74% of the stress and 82% of the strain to rupture. Multiple subfailures were evident in 79% of specimens. Present results supported the clinical theory that nonimmediate death as a result of blunt aortic injury is commonly caused by propagation of lesser lesions, initiating on the intimal layer, into complete vessel rupture including the adventitial layer. This finding, along with histologic evidence of subfailure pathological findings, confirms the presence of an acute window during which recognition and initiation of permissive hypotension may be lifesaving.

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

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Hashimoto, Junichiro; Ito, Sadayoshi

2015-07-01

Aortic stiffness determines the glomerular filtration rate (GFR) and predicts the progressive decline of the GFR. However, the underlying pathophysiological mechanism remains obscure. Recent evidence has shown a close link between aortic stiffness and the bidirectional (systolic forward and early diastolic reverse) flow characteristics. We hypothesized that the aortic stiffening-induced renal dysfunction is attributable to altered central flow dynamics. In 222 patients with hypertension, Doppler velocity waveforms were recorded at the proximal descending aorta to calculate the reverse/forward flow ratio. Tonometric waveforms were recorded to measure the carotid-femoral (aortic) and carotid-radial (peripheral) pulse wave velocities, to estimate the aortic pressure from the radial waveforms, and to compute the aortic characteristic impedance. In addition, renal hemodynamics was evaluated by duplex ultrasound. The estimated GFR was inversely correlated with the aortic pulse wave velocity, reverse/forward flow ratio, pulse pressure, and characteristic impedance, whereas it was not correlated with the peripheral pulse wave velocity or mean arterial pressure. The association between aortic pulse wave velocity and estimated GFR was independent of age, diabetes mellitus, hypercholesterolemia, and antihypertensive medication. However, further adjustment for the aortic reverse/forward flow ratio and pulse pressure substantially weakened this association, and instead, the reverse/forward flow ratio emerged as the strongest determinant of estimated GFR (P=0.001). A higher aortic reverse/forward flow ratio was also associated with lower intrarenal forward flow velocities. These results suggest that an increase in aortic flow reversal (ie, retrograde flow from the descending thoracic aorta toward the aortic arch), caused by aortic stiffening and impedance mismatch, reduces antegrade flow into the kidney and thereby deteriorates renal function. © 2015 American Heart Association

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

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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.

Primary Intra-aortic Epstein-Barr Virus-Positive Large B-Cell Lymphoma Presenting as Aortic Mural Thrombosis: An Entity Distinct From Intravascular Large B-Cell Lymphoma.

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Nakao, Ryuta; Sakashita, Aki; Omoto, Atsushi; Sato, Osamu; Hino, Yoko; Yanagisawa, Akio; Urata, Yoji

2017-12-01

Intravascular selective growth of neoplastic B lymphocytes is a characteristic finding of intravascular large B-cell lymphoma (IVLBCL). However, because neoplastic B cells of IVLBCL grow merely in the lumina of capillaries or small vessels, primary IVLBCL of the great vessels is considered exceptional. To our knowledge, only 2 primary B-cell lymphomas in the lumina of the vena cava have been reported. However, there has been no report of primary B-cell lymphoma with intra-aortic growth. We describe a novel manifestation of primary Epstein-Barr virus-positive large B-cell lymphoma mainly affecting the lumina of the aorta and its major branches in a 76-year-old man. He had a long-term fever that was refractory to antibiotics and aortic mural thrombosis with visceral embolization. Because he had no detectable mass suggesting a malignancy, it was difficult to diagnose while he was alive. He died without anticancer treatment, and the confirmed diagnosis was made at autopsy.

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

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

2013-01-01

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

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

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

2015-10-15

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

Anterior maxillary segmental distraction in the treatment of severe maxillary hypoplasia secondary to cleft lip and palate.

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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.

Bovine aortic arch with supravalvular aortic stenosis.

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Idhrees, Mohammed; Cherian, Vijay Thomas; Menon, Sabarinath; Mathew, Thomas; Dharan, Baiju S; Jayakumar, K

2016-09-01

A 5-year-old boy was diagnosed to have supravalvular aortic stenosis (SVAS). On evaluation of CT angiogram, there was associated bovine aortic arch (BAA). Association of BAA with SVAS has not been previously reported in literature, and to best of our knowledge, this is the first case report of SVAS with BAA. Recent studies show BAA as a marker for aortopathy. SVAS is also an arteriopathy. In light of this, SVAS can also possibly be a manifestation of aortopathy associated with BAA. Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

Sex Differences in Phenotypes of Bicuspid Aortic Valve and Aortopathy: Insights From a Large Multicenter, International Registry.

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Kong, William K F; Regeer, Madelien V; Ng, Arnold C T; McCormack, Louise; Poh, Kian Keong; Yeo, Tiong Cheng; Shanks, Miriam; Parent, Sarah; Enache, Roxana; Popescu, Bogdan A; Yip, James W; Ma, Lawrence; Kamperidis, Vasileios; van der Velde, Enno T; Mertens, Bart; Ajmone Marsan, Nina; Delgado, Victoria; Bax, Jeroen J

2017-03-01

This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta. New cases of endocarditis and aortic dissection were recorded. There were no significant sex differences regarding BAV morphology and frequency of normal valve function. When presenting with moderate/severe aortic valve dysfunction, men had more frequent aortic regurgitation than women (33.8% versus 22.2%, P <0.001), whereas women were more likely to have aortic stenosis (34.5% versus 44.1%, P <0.001). Men had more frequently isolated dilatation of the sinus of Valsalva or sinotubular junction (14.2% versus 6.7%, P <0.001) and diffuse dilatation of the aortic root and ascending aorta (16.2% versus 7.3%, P <0.001) than women. Endocarditis (4.5% versus 2.5%, P =0.037) and aortic dissections (0.5% versus 0%, P <0.001) occurred more frequently in men. Although there is a male predominance among patients with BAV, men with BAV had more frequently moderate/severe aortic regurgitation at first presentation compared with women, whereas women presented more often with moderate/severe aortic stenosis compared with men. Furthermore, men had more frequent aortopathy than women. © 2017 American Heart Association, Inc.

Aortopulmonary Fistula Presenting without an Endoleak after Thoracic Endovascular Aortic Repair

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Giacomo Sica

2017-08-01

Full Text Available Herein, we report the case of a 60-year-old man, a smoker with a history of arterial hypertension and diabetes mellitus. After computed tomography (CT for an episode of hemoptysis, the patient underwent elective thoracic endovascular aortic repair (TEVAR because of a degenerative aneurysm of the descending thoracic aorta. The area of perianeurysmal pulmonary atelectasis reported on the CT scan was not considered. Three months later, he developed an aortopulmonary fistula without endoleaks. Although TEVAR is a relatively safe procedure, no detail should be overlooked in the preoperative evaluation in order to avoid life-threatening complications. Further, the effectiveness and modality of prolonged antibiotic prophylaxis and/or preoperative respiratory physiotherapy should be assessed in such cases.

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

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Marcelo Cury

2013-01-01

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

Factors associated with the development of aortic valve regurgitation over time after two different techniques of valve-sparing aortic root surgery.

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Hanke, Thorsten; Charitos, Efstratios I; Stierle, Ulrich; Robinson, Derek; Gorski, Armin; Sievers, Hans-H; Misfeld, Martin

2009-02-01

Early results after aortic valve-sparing root reconstruction are excellent. Longer-term follow-up, especially with regard to aortic valve function, is required for further judgment of these techniques. Between July of 1993 and September of 2006, 108 consecutive patients (mean age 53.0 +/- 15.8 years) underwent the Yacoub operation (group Y) and 83 patients underwent the David operation (group D). Innovative multilevel hierarchic modeling methods were used to analyze aortic regurgitation over time. In general, aortic regurgitation increased with time in both groups. Factors associated with the development of a significant increase in aortic regurgitation were Marfan syndrome, concomitant cusp intervention, and preoperative aortic anulus dimension. In Marfan syndrome, the initial aortic regurgitation was higher in group Y versus group D (0.56 aortic regurgitation vs 0.29 aortic regurgitation, P = .049), whereas the mean annual progression rate of aortic regurgitation was marginally higher in group Y (0.132 aortic regurgitation vs 0.075 aortic regurgitation, P = .1). Concomitant cusp intervention was associated with a significant aortic regurgitation increase in both groups (P Marfan syndrome and a large preoperative aortic annulus diameter were better treated with the reimplantation technique, whereas those with a smaller diameter were better treated with the remodeling technique. Concomitant free-edge plication of prolapsing cusps was disadvantageous in both groups. Considering these factors may serve to improve the aortic valve longevity after valve-sparing aortic root surgery.

Presentation of an uncommon form of aortic dissection and rupture in Marifoan syndrome; Presentacion de una forma infecuente de diseccion y rotura aortica en el sindrome de Marfan

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Delgado, I; Ruiz, R; Villanueva, J M; Fernandez Cueto, J L [Servicio de Radiodiagnostico, Complejo Hospitalario, Ciudad Real (Spain)

1995-11-01

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

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

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

2018-04-01

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

Aortic valve surgery - open

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... gov/ency/article/007408.htm Aortic valve surgery - open To use the sharing features on this page, ... separates the heart and aorta. The aortic valve opens so blood can flow out. It then closes ...

Aortic annulus eccentricity before and after transcatheter aortic valve implantation: Comparison of balloon-expandable and self-expanding prostheses

International Nuclear Information System (INIS)

Schuhbaeck, Annika; Weingartner, Christina; Arnold, Martin; Schmid, Jasmin; Pflederer, Tobias; Marwan, Mohamed; Rixe, Johannes; Nef, Holger; Schneider, Christian; Lell, Michael; Uder, Michael; Ensminger, Stephan; Feyrer, Richard; Weyand, Michael; Achenbach, Stephan

2015-01-01

Highlights: • Post-implant geometry of catheter-based aortic valve prostheses is influenced by aortic valve calcification. • Balloon-expandable prostheses are more circular as compared to self-expanding prostheses. • The impact of post-implant geometry on valve function needs to be investigated. - Abstract: Introduction: The geometry of the aortic annulus and implanted transcatheter aortic valve prosthesis might influence valve function. We investigated the influence of valve type and aortic valve calcification on post-implant geometry of catheter-based aortic valve prostheses. Methods: Eighty consecutive patients with severe aortic valve stenosis (mean age 82 ± 6 years) underwent computed tomography before and after TAVI. Aortic annulus diameters were determined. Influence of prosthesis type and degree of aortic valve calcification on post-implant eccentricity were analysed. Results: Aortic annulus eccentricity was reduced in patients after TAVI (0.21 ± 0.06 vs. 0.08 ± 0.06, p < 0.0001). Post-TAVI eccentricity was significantly lower in 65 patients following implantation of a balloon-expandable prosthesis as compared to 15 patients who received a self-expanding prosthesis (0.06 ± 0.05 vs. 0.15 ± 0.07, p < 0.0001), even though the extent of aortic valve calcification was not different. After TAVI, patients with a higher calcium amount retained a significantly higher eccentricity compared to patients with lower amounts of calcium. Conclusions: Patients undergoing TAVI with a balloon-expandable prosthesis show a more circular shape of the implanted prosthesis as compared to patients with a self-expanding prosthesis. Eccentricity of the deployed prosthesis is affected by the extent of aortic valve calcification

Early Outcomes of Sutureless Aortic Valves

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Muhammet Onur Hanedan

2016-06-01

Full Text Available Background: In elderly high-risk surgical patients, sutureless aortic valve replacement (AVR should be an alternative to standard AVR. The potential advantages of sutureless aortic prostheses include reducing cross-clamping and cardiopulmonary bypass (CPB time and facilitating minimally invasive surgery and complex cardiac interventions, while maintaining satisfactory hemodynamic outcomes and low rates of paravalvular leakage. The current study reports our single-center experience regarding the early outcomes of sutureless aortic valve implantation. Methods: Between October 2012 and June 2015, 65 patients scheduled for surgical valve replacement with symptomatic aortic valve disease and New York Heart Association function of class II or higher were included to this study. Perceval S (Sorin Biomedica Cardio Srl, Sallugia, Italy and Edwards Intuity (Edwards Lifesciences, Irvine, CA, USA valves were used. Results: The mean age of the patients was 71.15±8.60 years. Forty-four patients (67.7% were female. The average preoperative left ventricular ejection fraction was 56.9±9.93. The CPB time was 96.51±41.27 minutes and the cross-clamping time was 60.85±27.08 minutes. The intubation time was 8.95±4.19 hours, and the intensive care unit and hospital stays were 2.89±1.42 days and 7.86±1.42 days, respectively. The mean quantity of drainage from chest tubes was 407.69±149.28 mL. The hospital mortality rate was 3.1%. A total of five patients (7.69% died during follow-up. The mean follow-up time was 687.24±24.76 days. The one-year survival rate was over 90%. Conclusion: In the last few years, several models of valvular sutureless bioprostheses have been developed. The present study evaluating the single-center early outcomes of sutureless aortic valve implantation presents the results of an innovative surgical technique, finding that it resulted in appropriate hemodynamic conditions with acceptable ischemic time.

Early Outcomes of Sutureless Aortic Valves.

Science.gov (United States)

Hanedan, Muhammet Onur; Mataracı, İlker; Yürük, Mehmet Ali; Özer, Tanıl; Sayar, Ufuk; Arslan, Ali Kemal; Ziyrek, Uğur; Yücel, Murat

2016-06-01

In elderly high-risk surgical patients, sutureless aortic valve replacement (AVR) should be an alternative to standard AVR. The potential advantages of sutureless aortic prostheses include reducing cross-clamping and cardiopulmonary bypass (CPB) time and facilitating minimally invasive surgery and complex cardiac interventions, while maintaining satisfactory hemodynamic outcomes and low rates of paravalvular leakage. The current study reports our single-center experience regarding the early outcomes of sutureless aortic valve implantation. Between October 2012 and June 2015, 65 patients scheduled for surgical valve replacement with symptomatic aortic valve disease and New York Heart Association function of class II or higher were included to this study. Perceval S (Sorin Biomedica Cardio Srl, Sallugia, Italy) and Edwards Intuity (Edwards Lifesciences, Irvine, CA, USA) valves were used. The mean age of the patients was 71.15±8.60 years. Forty-four patients (67.7%) were female. The average preoperative left ventricular ejection fraction was 56.9±9.93. The CPB time was 96.51±41.27 minutes and the cross-clamping time was 60.85±27.08 minutes. The intubation time was 8.95±4.19 hours, and the intensive care unit and hospital stays were 2.89±1.42 days and 7.86±1.42 days, respectively. The mean quantity of drainage from chest tubes was 407.69±149.28 mL. The hospital mortality rate was 3.1%. A total of five patients (7.69%) died during follow-up. The mean follow-up time was 687.24±24.76 days. The one-year survival rate was over 90%. In the last few years, several models of valvular sutureless bioprostheses have been developed. The present study evaluating the single-center early outcomes of sutureless aortic valve implantation presents the results of an innovative surgical technique, finding that it resulted in appropriate hemodynamic conditions with acceptable ischemic time.

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

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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).

Supravalvular aortic stenosis with sudden cardiac death

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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.

Aortic valve replacement and prosthesis-patient mismatch in the era of trans-catheter aortic valve implantation.

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Morita, Shigeki

2016-08-01

The treatment strategy for aortic stenosis (AS) has been changing due to newly developed valvular prostheses and trans-catheter aortic valve implantation (TAVI). To determine the role of new modalities for AS with a small aortic root, papers using the concept of prosthesis-patient mismatch (PPM) were reviewed. First, to determine the cut-off value of the indexed effective orifice area (IEOA) for defining PPM, the studies of surgical aortic valve replacement (SAVR) with a follow-up longer than 5 years and a patient number larger than 500 were reviewed. Second, the papers comparing TAVI and SAVR were reviewed. Furthermore, the prevalence of PPM was reviewed, with the addition of papers on aortic root enlargement, sutureless AVR, and aortic valve reconstruction with autologous pericardium. The results of the long-term survival after aortic valve replacement (AVR) have indicated that an IEOA less than 0.65 cm(2)/m(2) should be avoided in all cases, whereas the indications for patients with an IEOA between 065 and 0.85 cm(2)/m(2) should be determined by considering multiple factors. A large body size and younger age have a significantly negative influence on the long-term survival. In Asian population, the prevalence of PPM was low, despite the fact that the size of the aortic annulus was small. The IEOA after TAVI was larger than after surgical AVR in population-matched studies. To evaluate the role of TAVI and other modalities for a small aortic root, studies with a longer follow-up and larger volume are thus warranted.

Association of aortic and main left coronary aneurysms with severe aortic insufficiency in Takayasu’s arteritis

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Marcelo Ferraz de Freitas

2011-04-01

Full Text Available Takayasu’s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches. As a result of intimal fibroproliferation, segmental stenosis, occlusion, dilatation, and aneurysmal formation of the involved vessels may develop. It is an uncommon disease and usually affects young Asian female patients during the second and third decades of life. Coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding. We describe a case of a previously healthy 26-year-old Caucasian female whose Takayasu’s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency.

Association of aortic and main left coronary aneurysms with severe aortic insufficiency in Takayasu’s arteritis

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Schafranski, Marcelo Derbli; Ferraz de Freitas, Marcelo; Valladão de Carvalho, Marcelo

2011-01-01

Takayasu’s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches. As a result of intimal fibroproliferation, segmental stenosis, occlusion, dilatation, and aneurysmal formation of the involved vessels may develop. It is an uncommon disease and usually affects young Asian female patients during the second and third decades of life. Coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding. We describe a case of a previously healthy 26-year-old Caucasian female whose Takayasu’s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency. PMID:24765288

Thoracic aortic aneurysms and dissections: endovascular treatment.

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Baril, Donald T; Cho, Jae S; Chaer, Rabih A; Makaroun, Michel S

2010-01-01

The treatment of thoracic aortic disease has changed radically with the advances made in endovascular therapy since the concept of thoracic endovascular aortic repair was first described 15 years ago. Currently, there is a diverse array of endografts that are commercially available to treat the thoracic aorta. Multiple studies, including industry-sponsored and single-institution reports, have demonstrated excellent outcomes of thoracic endovascular aortic repair for the treatment of thoracic aortic aneurysms, with less reported perioperative morbidity and mortality in comparison with conventional open repair. Additionally, similar outcomes have been demonstrated for the treatment of type B dissections. However, the technology remains relatively novel, and larger studies with longer term outcomes are necessary to more fully evaluate the role of endovascular therapy for the treatment of thoracic aortic disease. This review examines the currently available thoracic endografts, preoperative planning for thoracic endovascular aortic repair, and outcomes of thoracic endovascular aortic repair for the treatment of both thoracic aortic aneurysms and type B aortic dissections. Mt Sinai J Med 77:256-269, 2010. (c) 2010 Mount Sinai School of Medicine.

Aortic valve function after bicuspidization of the unicuspid aortic valve.

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Aicher, Diana; Bewarder, Moritz; Kindermann, Michael; Abdul-Khalique, Hashim; Schäfers, Hans-Joachim

2013-05-01

Unicuspid aortic valve (UAV) anatomy leads to dysfunction of the valve in young individuals. We introduced a reconstructive technique of bicuspidizing the UAV. Initially we copied the typical asymmetry of a normal bicuspid aortic valve (BAV) (I), later we created a symmetric BAV (II). This study compared the hemodynamic function of the two designs of a bicuspidized UAV. Aortic valve function was studied at rest and during exercise in 28 patients after repair of UAV (group I, n = 8; group II, n = 20). There were no differences among the groups I and II with respect to gender, age, body size, or weight. All patients were in New York Heart Association class I. Six healthy adults served as control individuals. All patients were studied with transthoracic echocardiography between 4 and 65 months postoperatively. Systolic gradients were assessed by continuous wave Doppler while patients were at rest and exercising on a bicycle ergometer. Aortic regurgitation was grade I or less in all patients. Resting gradients were significantly elevated in group I compared with group II and control individuals (group I, peak 33.8 ± 7.8 mm Hg; mean 19.1 ± 5.4 mm Hg; group II, peak 15.8 ± 5.4, mean 8.2 ± 2.8 mm Hg; control individuals, peak 6.0 ± 1.6, mean 3.2 ± 0.8 mm Hg; p competence. A symmetric repair design leads to improved systolic aortic valve function at rest and during exercise. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Aortic annulus and ascending aorta: Comparison of preoperative and periooperative measurement in patients with aortic stenosis

International Nuclear Information System (INIS)

Smid, Michal; Ferda, Jiri; Baxa, Jan; Cech, Jakub; Hajek, Tomas; Kreuzberg, Boris; Rokyta, Richard

2010-01-01

Background: Precise determination of the aortic annulus size constitutes an integral part of the preoperative evaluation prior to aortic valve replacement. It enables the estimation of the size of prosthesis to be implanted. Knowledge of the size of the ascending aorta is required in the preoperative analysis and monitoring of its dilation enables the precise timing of the operation. Our goal was to compare the precision of measurement of the aortic annulus and ascending aorta using magnetic resonance (MR), multidetector-row computed tomography (MDCT), transthoracic echocardiography (TTE), and transoesophageal echocardiography (TEE) in patients with degenerative aortic stenosis. Methods and results: A total of 15 patients scheduled to have aortic valve replacement were enrolled into this prospective study. TTE was performed in all patients and was supplemented with TEE, CT and MR in the majority of patients. The values obtained were compared with perioperative measurements. For the measurement of aortic annulus, MR was found to be the most precise technique, followed by MDCT, TTE, and TEE. For the measurement of ascending aorta, MR again was found to be the most precise technique, followed by MDCT, TEE, and TTE. Conclusion: In our study, magnetic resonance was found to be the most precise technique for the measurement of aortic annulus and ascending aorta in patients with severe degenerative aortic stenosis.

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

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

2018-01-01

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

Aortic arch malformations

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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.)

Aortic arch malformations

International Nuclear Information System (INIS)

Kellenberger, Christian J.

2010-01-01

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

Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

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

2015-01-01

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

Aortic distensibility after aortic root replacement assessed with cardiovascular magnetic resonance.

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Melina, Giovanni; Rajappan, Kim; Amrani, Mohamed; Khaghani, Asghar; Pennell, Dudley J; Yacoub, Magdi H

2002-01-01

The changes in geometry of the aortic root during the cardiac cycle are thought to be essential for optimal valve function, both in terms of leaflet stress and dynamic behavior. Using cardiac magnetic resonance (CMR), the study aim was to determine aortic root distensibility of the homograft (group H) and the Medtronic Freestyle xenograft (group F) after aortic root replacement, from a prospective randomized trial. CMR was performed in 15 patients (six homografts, nine Freestyle) at six months and one year after surgery. Percentage change in aortic radius (PCR) and pressure strain elastic modulus (PSEM) were measured as indices of distensibility, and results related to left ventricular mass (LVM). At six months after surgery, mean PCR was 12+/-2.5 in group H and 12.9+/-6.1 in group F (p = NS), and PSEM was 428.5+/-69.8 and 493.5+/-72.7 g/cm2, respectively (p = NS). PCR was reduced to 10+/-1.7% in group H, and by 8.5+/-2.8% in group F (p = NS), while PSEM was increased to 520.5+/-87.3 and 825+/-420.4, respectively (p = NS) at the one-year follow up. Regression analysis showed a correlation between PCR and LVM (r = 0.52, p = 0.08) and LVM index (r = 0.46, p = 0.14), respectively. In addition, there was a relationship between PSEM, LVM and LVM index, suggesting that the stiffer the root wall, the higher the postoperative LVM. Up to one year after aortic root replacement, the wall of both the allogenic and xenogenic valves retained near-normal distensibility. For the first time, a correlation was demonstrated between the elastic properties of the aortic root and LVM. The longer-term behavior and clinical implications of these findings require further investigation.

Intra Aortic Balloon Pump (IABP: Past, Present and Future

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Jatin D Dedhia

2008-01-01

Full Text Available Intra Aortic Balloon Pump (IABP is the most commonly used mechanical circulatory assist device in cardiac patients. IABP can improve ventricular function by decreasing the preload and increasing systolic output with signifi-cant improvement in myocardial oxygen supply/demand ratio. Pre-operative IABP therapy in ′high-risk′ coronary patients has been shown to reduce hospital mortality and shorten ICU stay significantly, compared with controls. The introduction of sheathless insertion kits has reduced the incidence of vascular complication rates. Pro-Active Counter Pulsation is a new IABP system which automatically detects the irregular pattern and result is an improvement in the haemodynamic effect of IABP during periods of arrhythmia. Today, continued improvements in IABP technology permit safer use and earlier intervention to provide haemodynamic support. These developments have made the IABP a mainstay in the management of ischemic and dysfunctional myocardium. This review article aims to provide basic concept of IABP to hospital doctors especially anaesthetists, intensivists cardiologists and cardiac surgeons. It discusses the common indications, contraindications, the physiologic aspects of IABP, the equipment needed to facilitate IABP, the use of the IABP in special situations and complications of its use.

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

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

2016-08-01

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

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

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

2012-02-01

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

Abdominal Aortic Aneurysm (AAA)

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... Professions Site Index A-Z Abdominal Aortic Aneurysm (AAA) Abdominal aortic aneurysm (AAA) occurs when atherosclerosis or plaque buildup causes the ... weak and bulge outward like a balloon. An AAA develops slowly over time and has few noticeable ...

A History of Thoracic Aortic Surgery.

Science.gov (United States)

McFadden, Paul Michael; Wiggins, Luke M; Boys, Joshua A

2017-08-01

Ancient historical texts describe the presence of aortic pathology conditions, although the surgical treatment of thoracic aortic disease remained insurmountable until the 19th century. Surgical treatment of thoracic aortic disease then progressed along with advances in surgical technique, conduit production, cardiopulmonary bypass, and endovascular technology. Despite radical advances in aortic surgery, principles established by surgical pioneers of the 19th century hold firm to this day. Copyright © 2017 Elsevier Inc. All rights reserved.

Effects of acute dietary nitrate supplementation on aortic blood pressure and aortic augmentation index in young and older adults.

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Hughes, William E; Ueda, Kenichi; Treichler, David P; Casey, Darren P

2016-09-30

Aging is associated with elevated blood pressure (peripheral and aortic; BP) and aortic augmentation index (AIx) which may contribute to aortic BP. Although inorganic nitrate consumption reduces peripheral BP in both young and older adults, the effects of nitrate consumption on aortic BP and wave reflection in young and older adults is unknown. Therefore, we sought to characterize the effects of nitrate consumption on aortic BP and AIx in young and older adults. Noninvasive aortic pressure waveforms were synthesized from high-fidelity radial pressure waveforms via applanation tonometry before and following (60, 90, 120, 150, and 180 min) consumption of a nitrate-rich beetroot juice in 26 healthy adults (young: 25 ± 4 years, n = 14; older: 64 ± 5 years, n = 12). Aortic BP and indices of aortic wave reflection (AIx and AIx normalized for heart rate; AIx@75bpm) were calculated from the generated aortic pressure waveform. Nitrate consumption increased plasma nitrite in both groups 60-180 min following beetroot consumption (P young and older adults (P age groups. Conversely, indices of aortic wave reflection were reduced only in young adults following nitrate consumption (range of change from baseline over time: AIx@75bpm, -4.3 to -8.8%, P adults. Taken together, our results suggest that acute dietary nitrate supplementation reduces peripheral and aortic BP similarly in young and older adults despite differential effects on aortic AIx between age groups. Copyright © 2016 Elsevier Inc. All rights reserved.

Association of aortic valve calcification severity with the degree of aortic regurgitation after transcatheter aortic valve implantation.

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Koos, Ralf; Mahnken, Andreas Horst; Dohmen, Guido; Brehmer, Kathrin; Günther, Rolf W; Autschbach, Rüdiger; Marx, Nikolaus; Hoffmann, Rainer

2011-07-15

This study sought to examine a possible relationship between the severity of aortic valve calcification (AVC), the distribution of AVC and the degree of aortic valve regurgitation (AR) after transcatheter aortic valve implantation (TAVI) for severe aortic stenosis (AS). 57 patients (22 men, 81 ± 5 years) with symptomatic AS and with a logistic EuroSCORE of 24 ± 12 were included. 38 patients (67%) received a third (18F)-generation CoreValve® aortic valve prosthesis, in 19 patients (33%) an Edwards SAPIEN™ prosthesis was implanted. Prior to TAVI dual-source computed tomography for assessment of AVC was performed. To determine the distribution of AVC the percentage of the calcium load of the most severely calcified cusp was calculated. After TAVI the degree of AR was determined by angiography and echocardiography. The severity of AR after TAVI was related to the severity and distribution of AVC. There was no association between the distribution of AVC and the degree of paravalvular AR after TAVI as assessed by angiography (r = -0.02, p = 0.88). Agatston AVC scores were significantly higher in patients with AR grade ≥ 3 (5055 ± 1753, n = 3) than in patients with AR grade AVC scores > 3000 were associated with a relevant paravalvular AR and showed a trend for increased need for second manoeuvres. There was a significant correlation between the severity of AVC and the degree of AR after AVR (r = 0.50, p AVC have an increased risk for a relevant AR after TAVI as well as a trend for increased need for additional procedures. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

Aortic valve replacement for Libman-Sacks endocarditis.

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Keenan, Jack B; Janardhanan, Rajesh; Larsen, Brandon T; Khalpey, Zain

2016-10-04

A 24-year-old man with systemic lupus erythematosus and antiphospholipid syndrome complicated by lupus nephritis presented with acute limb ischaemia secondary to an embolus. Following embolectomy, the patient underwent a transthoracic echocardiogram which revealed a large vegetation on all three cusps of the aortic valve. The patient was taken for an urgent aortic valve replacement with a mechanical valve. Cultures of one cusp remained sterile. Histopathological examination of the remaining two cusps revealed sterile fibrin-rich thrombotic vegetations characteristic of non-bacterial thrombotic endocarditis. 2016 BMJ Publishing Group Ltd.

Infective endocarditis following transcatheter aortic valve replacement-

DEFF Research Database (Denmark)

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

2013-01-01

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

Liver segment IV hypoplasia as a risk factor for bile duct injury.

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Mercado, Miguel Angel; Franssen, Bernardo; Arriola, Juan Carlos; Garcia-Badiola, Artemio; Arámburo, Rigoberto; Elnecavé, Alejandro; Cortés-González, Rubén

2011-09-01

Bile duct injury remains constant in the era of laparoscopic cholecystectomy and misidentification of structures remains one of the most common causes of such injuries. Abnormalities in liver segment IV, which is fully visible during laparoscopic cholecystectomy, may contribute to misidentification as proposed herein. We describe the case of a 36-year-old female who had a bile duct injury during a laparoscopic cholecystectomy where the surgeon noticed an unusually small distance between the gallbladder and the round ligament. We define hypoplasia of liver segment IV as well as describe the variation of the biliary anatomy in the case. We also intend to fit it in a broader spectrum of developmental anomalies that have both hyopoplasia of some portion of the liver and variations in gallbladder and bile duct anatomy that may contribute to bile duct injury. To our knowledge, hypoplasia of liver segment IV has not been suggested in the literature as a risk factor for bile duct injury except in the extreme case of a left-sided gallbladder. Surgeons should be vigilant during laparoscopic cholecystectomy when they become aware of an unusually small distance between the gallbladder bed and the round ligament prior to beginning their dissection, variations in the common bile duct and cystic duct should be expected.

Is there a rural gradient in the diagnosis of aortic stenosis? An analysis of a remote Scottish cohort.

Science.gov (United States)

Maycock, Matthew I; Farman, Colin; Mort, Alasdair; Turpie, David; Leslie, Stephen J

2013-01-01

Calcific aortic stenosis is the most common cardiac valve lesion and is becoming increasingly prevalent as life expectancy rises. There is evidence that patients in remote and rural areas with certain diseases have worse outcomes and present to specialist services later than their urban counterparts. It is not known whether patients with aortic stenosis follow a similar pattern. The aim of this study was to investigate whether increasing rurality was associated with later presentation to healthcare services at a more advanced stage of aortic stenosis. This was a retrospective cohort study. Using ICD-10 discharge codes and local databases, 605 patients with aortic stenosis who presented between 31 November 1999 and 1 December 2008 were identified. Aortic stenosis was defined as a pressure gradient across the aortic valve of 25 mmHg or more. Patients with prior aortic valve replacement were excluded. Clinical notes were reviewed for all patients. Gender, age and pressure gradient across the aortic valve at presentation and patient GP-practice location were recorded. Patients were then assigned a Clinical Peripherality Index score based on the postcode of their GP's practice to define rurality. Patient data were compared across the six defined levels of clinical peripherality by ANOVA. Mean patient age was 73 ± 13 years, and 336 (54%) were male. The peak gradient across the valve was 41.1 ± 26.7 mmHg. There was no association between the level of clinical peripherality and the stage of aortic stenosis at presentation, age or gender (all p >0.05). There was no urban-rural gradient in the severity of aortic stenosis at presentation in this remote Scottish cohort. This suggests that patients with this condition in remote areas do not present later in their disease trajectory.

Robotic aortic surgery.

Science.gov (United States)

Duran, Cassidy; Kashef, Elika; El-Sayed, Hosam F; Bismuth, Jean

2011-01-01

Surgical robotics was first utilized to facilitate neurosurgical biopsies in 1985, and it has since found application in orthopedics, urology, gynecology, and cardiothoracic, general, and vascular surgery. Surgical assistance systems provide intelligent, versatile tools that augment the physician's ability to treat patients by eliminating hand tremor and enabling dexterous operation inside the patient's body. Surgical robotics systems have enabled surgeons to treat otherwise untreatable conditions while also reducing morbidity and error rates, shortening operative times, reducing radiation exposure, and improving overall workflow. These capabilities have begun to be realized in two important realms of aortic vascular surgery, namely, flexible robotics for exclusion of complex aortic aneurysms using branched endografts, and robot-assisted laparoscopic aortic surgery for occlusive and aneurysmal disease.

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

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Chii-Shyan Lay

2006-07-01

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

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

Science.gov (United States)

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

2002-07-01

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

Diagnostic imaging of acute aortic dissection

International Nuclear Information System (INIS)

Ohya, Tohru; Kumazaki, Tatsuo

1991-01-01

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

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

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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%

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

International Nuclear Information System (INIS)

Drozd, V.; Danilova, L.; Lushchyk, M.; Leonova, T.; Platonova, T.; Grigorovich, A.; Sivuda, V.; Branovan, I.; Biko, I.; Reiners, C.

2012-01-01

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%

Bicuspid aortic valves: Diagnostic accuracy of standard axial 64-slice chest CT compared to aortic valve image plane ECG-gated cardiac CT

Energy Technology Data Exchange (ETDEWEB)

Murphy, David J., E-mail: david.murphy@st-vincents.ie [Department of Radiology, St Vincent' s University Hospital, Elm Park, Dublin 4 (Ireland); McEvoy, Sinead H., E-mail: s.mcevoy@st-vincents.ie [Department of Radiology, St Vincent' s University Hospital, Elm Park, Dublin 4 (Ireland); Iyengar, Sri, E-mail: sri.iyengar@nhs.net [Department of Radiology, Plymouth Hospitals NHS Trust, Plymouth Devon PL6 8DH (United Kingdom); Feuchtner, Gudrun, E-mail: Gudrun.Feuchtner@i-med.ac.at [Department of Radiology, Innsbruck Medical University, Anichstr. 35, A-6020 Innsbruck (Austria); Cury, Ricardo C., E-mail: r.cury@baptisthealth.net [Department of Radiology, Baptist Cardiac and Vascular Institute, 8900 North Kendall Drive, Miami, FL 33176 (United States); Roobottom, Carl, E-mail: carl.roobottom@nhs.net [Department of Radiology, Plymouth Hospitals NHS Trust, Plymouth Devon PL6 8DH (United Kingdom); Plymouth University Peninsula Schools of Medicine and Dentistry (United Kingdom); Baumueller, Stephan, E-mail: Hatem.Alkadhi@usz.ch [Institute for Diagnostic and Interventional Radiology, University Hospital Zurich, Raemistrasse 100, CH-8091 Zurich (Switzerland); Alkadhi, Hatem, E-mail: stephan.baumueller@usz.ch [Institute for Diagnostic and Interventional Radiology, University Hospital Zurich, Raemistrasse 100, CH-8091 Zurich (Switzerland); Dodd, Jonathan D., E-mail: jonniedodd@gmail.com [Department of Radiology, St Vincent' s University Hospital, Elm Park, Dublin 4 (Ireland)

2014-08-15

Objectives: To assess the diagnostic accuracy of standard axial 64-slice chest CT compared to aortic valve image plane ECG-gated cardiac CT for bicuspid aortic valves. Materials and methods: The standard axial chest CT scans of 20 patients with known bicuspid aortic valves were blindly, randomly analyzed for (i) the appearance of the valve cusps, (ii) the largest aortic sinus area, (iii) the longest aortic cusp length, (iv) the thickest aortic valve cusp and (v) valve calcification. A second blinded reader independently analyzed the appearance of the valve cusps. Forty-two age- and sex-matched patients with known tricuspid aortic valves were used as controls. Retrospectively ECG-gated cardiac CT multiphase reconstructions of the aortic valve were used as the gold-standard. Results: Fourteen (21%) scans were scored as unevaluable (7 bicuspid, 7 tricuspid). Of the remainder, there were 13 evaluable bicuspid valves, ten of which showed an aortic valve line sign, while the remaining three showed a normal Mercedes-Benz appearance owing to fused valve cusps. The 35 evaluable tricuspid aortic valves all showed a normal Mercedes-Benz appearance (P = 0.001). Kappa analysis = 0.62 indicating good interobserver agreement for the aortic valve cusp appearance. Aortic sinus areas, aortic cusp lengths and aortic cusp thicknesses of ≥3.8 cm{sup 2}, 3.2 cm and 1.6 mm respectively on standard axial chest CT best distinguished bicuspid from tricuspid aortic valves (P < 0.0001 for all). Of evaluable scans, the sensitivity, specificity, positive and negative predictive values of standard axial chest CT in diagnosing bicuspid aortic valves was 77% (CI 0.54–1.0), 100%, 100% and 70% respectively. Conclusion: The aortic valve is evaluable in approximately 80% of standard chest 64-slice CT scans. Bicuspid aortic valves may be diagnosed on evaluable scans with good diagnostic accuracy. An aortic valve line sign, enlarged aortic sinuses and elongated, thickened valve cusps are specific CT

Aortic aneurysm disease vs. aortic occlusive disease - differences in outcome and intensive care resource utilisation after elective surgery

DEFF Research Database (Denmark)

Bisgaard, Jannie; Gilsaa, Torben; Rønholm, Ebbe

2013-01-01

clamping is more pronounced in patients with aortic aneurysm disease, which may affect outcome. OBJECTIVES: The aim of this observational cohort study was to evaluate outcome after open elective abdominal aortic surgery, hypothesising a higher 30-day mortality, a higher incidence of postoperative organ...... dysfunction and a longer length of stay in patients with aortic aneurysm compared with aortic occlusive disease. DESIGN: Cohort observational study based on prospective registrations from national databases. SETTING: Eight Danish hospitals, including four university and four non-university centres, from 1...... or inotropes, ICU stay more than 24 h, hospital length of stay and mortality. RESULTS: Compared with aortic occlusive disease, more patients with aortic aneurysm disease had ICU stays more than 24 h (62 vs. 45%, P ...

MRI evaluation of the aortic disease

International Nuclear Information System (INIS)

Kapuscinski, O.; Polkowski, J.; Zaleska, T.; Walecki, J.; Biesiadko, M.

1994-01-01

The goal of our study was to establish the value of MRI in diagnosing aortic disease. During 3 years period 46 patients were examined (12 women aged from 6 to 62 years and 34 men aged from 8 to 72 years). In 28 cases the thoracic aorta was examined, in 6 cases the abdominal aorta and in 12 - both the thoracic and the abdominal aorta. MR images were performed on MRT 50 A Toshiba unit 0.5 T. MR images demonstrated aortic aneurysm (caused by atherosclerosis or trauma), aortic dissections, aortic anomalies (i.e. in Turner syndrome) and non-specific aortitis and vasculitis. We regard MRI as preferable noninvasive imaging technique in diagnosing aortic disease. (author)

Aortic allografts in treatment of aortic valve and ascending aorta prosthetic endocarditis

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S.V. Spiridonov

2017-03-01

Full Text Available The aim – to assess short- and long-term results of aortic root replacement using aortic allografts in patients with prosthetic endocarditis. Materials and methods. Since February 2009 until June 2016 aortic valve and ascending aorta replacement using aortic allografts was performed in 26 patients with prosthetic endocarditis. In 50 % of cases at initial operation aortic valve replacement was performed, in another 50 % of cases – aortic valve and ascending aorta replacement. Echocardiography was performed 10 days, 3, 6 and 12 months, 2, 3 and 5 years after surgery. Analysis of long-term results included all cases of deaths, prosthesis-related complications and recurrence of endocarditis. Results. 30-day mortality was 23.1 %. Extracorporeal membranous oxygenation (ECMO was used only in 5 patients (19.2 %. Four patients were weaned from ECMO. We did not observe any allograft-related complications. During follow-up period there were no cases of reoperation due to structural allograft failure. Relapse of infection occurred in 1 patient (3.8 % four years after the operation and led to lethal outcome. Conclusion. Reoperations using allografts are an effective surgical treatment of prosthetic endocarditis. In majority of cases prosthetic endocarditis was caused by gram-positive cocci (Staphylococcus. In 84.6 % of cases it was associated with destruction of paravalvular structures and abscesses formation. Heart failure was a causative factor of different complications in these patients, which required ECMO in 19.2 % of patients. In 80 % of cases patients were weaned from ECMO. Allografts using for the treatment of prosthetic endocarditis is associated with high resistance to infection and with a significant rate of freedom from recurrence of endocarditis within 3 years after surgery.

Trans-sinusal maxillary distraction for correction of midfacial hypoplasia: long-term clinical results.

Science.gov (United States)

Nadjmi, N; Schutyser, F; Van Erum, R

2006-10-01

Maxillary distraction osteogenesis is indicated in severe angle class III malocclusions, and severe maxillary hypoplasia among some cleft patients and other craniofacial deformities. Twenty patients, aged 8-48 years (mean 17.8+/-10.5 SD) with maxillary and midfacial hypoplasia were treated. The follow-up period was 13-65 months (mean 35+/-16.3 SD). A trans-sinusal maxillary distractor was placed intraorally at each side of the maxilla. The distraction vector was predicted using specialist software, and was transferred to the patients using stereolithographic models and individual templates. A (high) Le Fort I type osteotomy was performed. The amount of activation varied from 8 to 17.5 mm (mean 13.1+/-2.9 SD). Soft and hard tissue formation resulted in complete healing across the distraction gaps. The distractors are almost completely submerged, and can be left in place as long as necessary to avoid relapse. Wit's appraisal was used to measure the stability of the long-term distraction results. Results up to 5 years after distraction showed considerable maxillary advancement with long-term stability. Ongoing growth of the facial skeleton must be considered when distraction osteogenesis is chosen in growing patients.

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.

Overexpression of interleukin-1β and interferon-γ in type I thoracic aortic dissections and ascending thoracic aortic aneurysms: possible correlation with matrix metalloproteinase-9 expression and apoptosis of aortic media cells.

Science.gov (United States)

Zhang, Lei; Liao, Ming-fang; Tian, Lei; Zou, Si-li; Lu, Qing-sheng; Bao, Jun-min; Pei, Yi-fei; Jing, Zai-ping

2011-07-01

To examine the expression of interleukin-1β and interferon-γ and their possible roles in aortic dissections and aneurysms. Aortic specimens were obtained from patients with type I thoracic aortic dissection, ascending thoracic aortic aneurysms, and control organ donors. The expression of interleukin-1β, interferon-γ, matrix metalloproteinase-9, and signal transduction factors phospho-p38 and phosphorylated c-jun N-terminal kinase (phospho-JNK) were detected by real time reverse transcription-polymerase chain reaction (real time RT-PCR), Western blot, and immunohistochemistry, respectively. Terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL) staining was performed to detect apoptosis of media cells. The correlation of these factors and apoptosis was also studied. Apoptosis in the media of thoracic aortic dissection and in ascending thoracic aortic aneurysms was dramatically higher than in the control group. The expression of interleukin-1β gradually increased from the control group, thoracic aortic dissection to ascending thoracic aortic aneurysms (p matrix metalloproteinase-9 was significantly increased in the media of thoracic aortic dissection and ascending thoracic aortic aneurysms compared with the control group (p correlations between interleukin-1β versus matrix metalloproteinase-9, interleukin-1β versus phospho-p38 in thoracic aortic dissection (p matrix metalloproteinase-9, interferon-γ versus phospho-JNK, interferon-γ versus apoptosis, and interleukin-1β versus apoptosis in ascending thoracic aortic aneurysms (p = 0.02, 0.02, p matrix metalloproteinase-9 and the apoptosis of media cells in humans. Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

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

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Francisco Diniz Affonso da Costa

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

The JUPITER registry: One-year outcomes of transapical aortic valve implantation using a second generation transcatheter heart valve for aortic regurgitation.

Science.gov (United States)

Silaschi, Miriam; Conradi, Lenard; Wendler, Olaf; Schlingloff, Friederike; Kappert, Utz; Rastan, Ardawan J; Baumbach, Hardy; Holzhey, David; Eichinger, Walter; Bader, Ralf; Treede, Hendrik

2018-06-01

We present 1-year outcomes of the post-market registry of a next-generation transcatheter heart valve used for aortic regurgitation (AR). Transcatheter aortic valve replacement (TAVR) is routine in high-risk patients with aortic stenosis but is not recommended for AR. The JenaValve™ (JenaValve Technology GmbH, Munich, Germany) overcomes technical challenges in AR patients through a leaflet clipping mechanism. The JenaValve EvalUation of Long Term Performance and Safety In PaTients with SEvere Aortic Stenosis oR Aortic Insufficiency (JUPITER) Registry is a European study to evaluate safety and effectiveness of this THV. From 2012-2015, 30 patients with AR were enrolled. Mean age was 74.4 ± 9.3 years. Procedural success was 96.7% (29/30). One patient was converted to open surgery. No annular rupture or coronary ostia obstruction occurred. Mortality at 30 days was 10.0% (3/30). Combined safety endpoint was met in 13.3% (4/30). Paravalvular regurgitation was not present/trivial in 84.6% (22/26) and mild in 15.4% (4/26). Rate of permanent pacemaker implantation was 3.8% (1/26). One-year Kaplan-Meier survival was 79.9%, one-year combined efficacy was 73.1% (19/30). No further strokes were observed during 1 year of follow-up. The JenaValve overcomes technical challenges of TAVR in AR through a clipping mechanism. We report satisfactory outcomes of a multicenter registry using the JenaValve for predominant AR, as rate of THV embolization, residual AR and permanent pacemaker implantation was low. One-year results using the JenaValve for AR encourage its use for this indication. © 2017 Wiley Periodicals, Inc.

A geometric reappraisal of proximal landing zones for thoracic endovascular aortic repair according to aortic arch types

NARCIS (Netherlands)

Marrocco-Trischitta, Massimiliano M.; de Beaufort, Hector W.; Secchi, Francesco; van Bakel, Theodorus M.; Ranucci, Marco; van Herwaarden, Joost A.; Moll, Frans L.; Trimarchi, Santi

Objective: This study assessed whether the additional use of the aortic arch classification in type I, II, and III may complement Ishimaru's aortic arch map and provide valuable information on the geometry and suitability of proximal landing zones for thoracic endovascular aortic repair. Methods:

Aortic elongation in aortic aneurysm and dissection: the Tübingen Aortic Pathoanatomy (TAIPAN) project.

Science.gov (United States)

Krüger, Tobias; Sandoval Boburg, Rodrigo; Lescan, Mario; Oikonomou, Alexandre; Schneider, Wilke; Vöhringer, Luise; Lausberg, Henning; Bamberg, Fabian; Blumenstock, Gunnar; Schlensak, Christian

2018-01-24

To study the lengths and diameters of aortic segments in healthy and diseased aortas and to assess the role of aortic elongation in Type A aortic dissection (TAD) prediction. Ectasia and aneurysm were defined by ascending aorta diameters of 45-54 mm and ≥55 mm, respectively. Computed tomography angiography studies of 256 healthy, 102 ectasia, 38 aneurysm, 17 pre-TAD and 166 TAD aortas were analysed using curved multiplanar reformats. The study groups were structurally equal. The diameter of the ascending aorta was 35 mm in the control group and was larger (P TAD (43 mm) and TAD (56 mm) groups. The length of the ascending aorta from the aortic annulus to the brachiocephalic trunk was 92 mm in the control group, 113 mm in the ectasia group, 120 mm in the aneurysm group and 111 mm and 118 mm in the pre-TAD and TAD groups (all P TAD group and 48% of the TAD group. The correlation between the diameter and the length of the ascending aorta was r = 0.752; therefore, both parameters must be examined separately. A score considering both parameters identified 23.5% of pre-TAD patients, significantly more than the diameter alone, and 31.4% of ectasia aortas were elongated. Patients with ectatic (45-54 mm diameter) and elongated (≥120 mm) ascending aortas represent a high-risk subpopulation for TAD. © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Mutations in X-linked PORCN, a putative regulator of Wnt signaling, cause focal dermal hypoplasia

Science.gov (United States)

Focal dermal hypoplasia is an X-linked dominant disorder characterized by patchy hypoplastic skin and digital, ocular, and dental malformations. We used array comparative genomic hybridization to identify a 219-kb deletion in Xp11.23 in two affected females. We sequenced genes in this region and fou...

Molecular and cellular mechanisms of aortic stenosis.

Science.gov (United States)

Yetkin, Ertan; Waltenberger, Johannes

2009-06-12

Calcific aortic stenosis is the most common cause of aortic valve replacement in developed countries, and this condition increases in prevalence with advancing age. The fibrotic thickening and calcification are common eventual endpoint in both non-rheumatic calcific and rheumatic aortic stenoses. New observations in human aortic valves support the hypothesis that degenerative valvular aortic stenosis is the result of active bone formation in the aortic valve, which may be mediated through a process of osteoblast-like differentiation in these tissues. Additionally histopathologic evidence suggests that early lesions in aortic valves are not just a disease process secondary to aging, but an active cellular process that follows the classical "response to injury hypothesis" similar to the situation in atherosclerosis. Although there are similarities with the risk factor and as well as with the process of atherogenesis, not all the patients with coronary artery disease or atherosclerosis have calcific aortic stenosis. This review mainly focuses on the potential vascular and molecular mechanisms involved in the pathogenesis of aortic valve stenosis. Namely extracellular matrix remodeling, angiogenesis, inflammation, and eventually osteoblast-like differentiation resulting in bone formation have been shown to play a role in the pathogenesis of calcific aortic stenosis. Several mediators related to underlying mechanisms, including growth factors especially transforming growth factor-beta1 and vascular endothelial growth factors, angiogenesis, cathepsin enzymes, adhesion molecules, bone regulatory proteins and matrix metalloproteinases have been demonstrated, however the target to be attacked is not defined yet.

Turner Syndrome in Girls Presenting with Coarctation of the Aorta.

Science.gov (United States)

Eckhauser, Aaron; South, Sarah T; Meyers, Lindsay; Bleyl, Steven B; Botto, Lorenzo D

2015-11-01

To evaluate the frequency of Turner syndrome in a population-based, statewide cohort of girls with coarctation of the aorta. The Utah Birth Defects Network was used to ascertain a cohort of girls between 1997 and 2011 with coarctation of the aorta. Livebirths with isolated coarctation of the aorta or transverse arch hypoplasia were included and patients with complex congenital heart disease not usually seen in Turner syndrome were excluded. Of 244 girls with coarctation of the aorta, 77 patients were excluded, leaving a cohort of 167 girls; 86 patients (51%) had chromosomal studies and 21 (12.6%) were diagnosed with Turner syndrome. All patients were diagnosed within the first 4 months of life and 5 (24%) were diagnosed prenatally. Fifteen patients (71%) had Turner syndrome-related findings in addition to coarctation of the aorta. Girls with mosaicism were less likely to have Turner syndrome-associated findings (3/6 mosaic girls compared with 12/17 girls with non-mosaic 45,X). Twelve girls (57%) diagnosed with Turner syndrome also had a bicommissural aortic valve. At least 12.6% of girls born with coarctation of the aorta have karyotype-confirmed Turner syndrome. Such a high frequency, combined with the clinical benefits of an early diagnosis, supports genetic screening for Turner syndrome in girls presenting with coarctation of the aorta. Copyright © 2015 Elsevier Inc. All rights reserved.

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

Science.gov (United States)

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

2008-01-01

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

Influence of the Quantity of Aortic Valve Calcium on the Agreement Between Automated 3-Dimensional Transesophageal Echocardiography and Multidetector Row Computed Tomography for Aortic Annulus Sizing.

Science.gov (United States)

Podlesnikar, Tomaz; Prihadi, Edgard A; van Rosendael, Philippe J; Vollema, E Mara; van der Kley, Frank; de Weger, Arend; Ajmone Marsan, Nina; Naji, Franjo; Fras, Zlatko; Bax, Jeroen J; Delgado, Victoria

2018-01-01

Accurate aortic annulus sizing is key for selection of appropriate transcatheter aortic valve implantation (TAVI) prosthesis size. The present study compared novel automated 3-dimensional (3D) transesophageal echocardiography (TEE) software and multidetector row computed tomography (MDCT) for aortic annulus sizing and investigated the influence of the quantity of aortic valve calcium (AVC) on the selection of TAVI prosthesis size. A total of 83 patients with severe aortic stenosis undergoing TAVI were evaluated. Maximal and minimal aortic annulus diameter, perimeter, and area were measured. AVC was assessed with computed tomography. The low and high AVC burden groups were defined according to the median AVC score. Overall, 3D TEE measurements slightly underestimated the aortic annulus dimensions as compared with MDCT (mean differences between maximum, minimum diameter, perimeter, and area: -1.7 mm, 0.5 mm, -2.7 mm, and -13 mm 2 , respectively). The agreement between 3D TEE and MDCT on aortic annulus dimensions was superior among patients with low AVC burden (AVC burden (≥3,025 arbitrary units). The interobserver variability was excellent for both methods. 3D TEE and MDCT led to the same prosthesis size selection in 88%, 95%, and 81% of patients in the total population, the low, and the high AVC burden group, respectively. In conclusion, the novel automated 3D TEE imaging software allows accurate and highly reproducible measurements of the aortic annulus dimensions and shows excellent agreement with MDCT to determine the TAVI prosthesis size, particularly in patients with low AVC burden. Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights reserved.

MRI and three dimensional ultrasonography in the assessment of pulmonary hypoplasia in fetuses with urinary tract anomalies

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Mariam Raafat

2016-12-01

Conclusion: There is a good concordance between 3D-US and MRI in the evaluation of PH in fetuses with UTM. MRI could be reserved for borderline cases of pulmonary hypoplasia and the difficult diagnostic situations.

Myocardial ischemia in severe aortic regurgitation despite angiographically normal coronary arteries

International Nuclear Information System (INIS)

Aksoy, S.; Cam, N.; Guney, M.R.; Gurkan, U.; Oz, D.; Poyraz, E.; Eksik, A.; Agirbasli, M.

2012-01-01

Patients with severe aortic regurgitation frequently present with angina pectoris. The exact pathophysiology for angina in aortic regurgitation is not clear. Left ventricular hypertrophy and myocardial blood supply-demand mismatch have been the suggested mechanisms to explain ischemia. However, no conclusive clinical study exists to define the incidence of ischemia in patients with severe aortic regurgitation and normal coronary arteries. We, therefore, investigated the frequency of myocardial ischemia in relation to left ventricular hypertrophy or dilatation in patients with severe aortic regurgitation and normal coronary arteries. We reviewed the medical records of all patients (n=311) with aortic valve replacement due to aortic regurgitation between 2007 and 2010. We selected subjects with normal coronary arteries (n=182) for the study purpose, and we identified 35 patients who underwent myocardial perfusion scintigraphy prior to the coronary angiography (19 female and 16 male subjects; age 45.0±8.9 years). Left ventricular hypertrophy and dilatation were detected in 9 (26%) and 5 (14%) patients, respectively. Myocardial perfusion scintigraphy showed evidence of ischemia in 10 (29%) patients with normal coronary arteries. The presence of ischemia did not relate to the presence of left ventricular hypertrophy and/or dilatation. As a potential mechanism, aortic regurgitation causes backflow of blood from the aorta into the left ventricle, hence disturbs coronary flow dynamics. In conclusion, myocardial ischemia is common (nearly one-third) among patients with severe aortic regurgitation even in the absence of coronary obstruction, left ventricular hypertrophy and/or dilatation. (author)

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.

Relationship between aortic diseases and renal cysts

International Nuclear Information System (INIS)

Hashizume, Toshikazu

2009-01-01

Based on empirical observations, patients with aortic diseases (AoD) (abdominal aortic aneurysm (AAA), thoracic aortic aneurysm (TAA) and aortic dissection (AD)) appeared to present with an increased incidence of renal cysts (RC) observed on computed tomography (CT). To clarify any potential relationship, I compared incidence of RC on CT scans in patients with and without AoD. A comparison was conducted on the incidence of RC on CT scans in 107 patients (74.6 years old; n=71 males, n=36 females) with AoD (57 AAA, 36 TAA, 14 AD) versus 332 control patients (73.6 years old; n=193 males, n=139 females) without AoD. Univariate analysis and multiple logistic regression were performed to evaluate the relationship between AoD and RC incidence. In patients with AoD, 65.0% presented with RC compared to only 28.6% in the control group (p<0.0001). In comparison, the AoD group presented with a higher frequency of hypertension (63.6% vs. 33.4%, p<0.0001), coronary artery disease (26.2% vs. 13.6%, p<0.0001), chronic obstructive pulmonary disease (COPD) (10.3% vs. 4.2%, p<0.0001), but no significant frequency of dyslipidemia and diabetes mellitus was observed between the 2 groups. Multivariate analysis indicated three independent predictors of AoD: hypertension (p=0.013; odds ratio=2.32), COPD (p=0.015; odds ratio=5.62) and RC (p<0.0001; odds ratio=4.88). These results indicate a significantly higher incidence of RC in patients with versus without AoD. A close relationship between AoD and RC may exist, and coincidental RC could be a potential indicator for AoD screening. (author)

Relationship between aortic diseases and renal cysts

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Hashizume, Toshikazu [Minami Wakayama Medical Center, Tanabe, Wakayama (Japan)

2009-12-15

Based on empirical observations, patients with aortic diseases (AoD) (abdominal aortic aneurysm (AAA), thoracic aortic aneurysm (TAA) and aortic dissection (AD)) appeared to present with an increased incidence of renal cysts (RC) observed on computed tomography (CT). To clarify any potential relationship, I compared incidence of RC on CT scans in patients with and without AoD. A comparison was conducted on the incidence of RC on CT scans in 107 patients (74.6 years old; n=71 males, n=36 females) with AoD (57 AAA, 36 TAA, 14 AD) versus 332 control patients (73.6 years old; n=193 males, n=139 females) without AoD. Univariate analysis and multiple logistic regression were performed to evaluate the relationship between AoD and RC incidence. In patients with AoD, 65.0% presented with RC compared to only 28.6% in the control group (p<0.0001). In comparison, the AoD group presented with a higher frequency of hypertension (63.6% vs. 33.4%, p<0.0001), coronary artery disease (26.2% vs. 13.6%, p<0.0001), chronic obstructive pulmonary disease (COPD) (10.3% vs. 4.2%, p<0.0001), but no significant frequency of dyslipidemia and diabetes mellitus was observed between the 2 groups. Multivariate analysis indicated three independent predictors of AoD: hypertension (p=0.013; odds ratio=2.32), COPD (p=0.015; odds ratio=5.62) and RC (p<0.0001; odds ratio=4.88). These results indicate a significantly higher incidence of RC in patients with versus without AoD. A close relationship between AoD and RC may exist, and coincidental RC could be a potential indicator for AoD screening. (author)

[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.

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

DEFF Research Database (Denmark)

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

2015-01-01

Surgical aortic valve replacement (SAVR) and, more recently, transcatheter aortic valve implantation (TAVI) have been shown to be the only treatments that can improve the natural cause of severe aortic valve stenosis. However, after SAVR and TAVI, the incidence of new-onset atrial fibrillation...... (NOAF) is 31%-64% and 4%-32%, respectively. NOAF is independently associated with adverse events such as stroke, death, and increased length of hospital stay. Increasing the knowledge of predisposing factors, optimal postprocedural monitoring, and prophylactic antiarrhythmic and antithrombotic therapy...

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

DEFF Research Database (Denmark)

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

2015-01-01

Surgical aortic valve replacement (SAVR) and, more recently, transcatheter aortic valve implantation (TAVI) have been shown to be the only treatments that can improve the natural cause of severe aortic valve stenosis. However, after SAVR and TAVI, the incidence of new-onset atrial fibrillation...... (NOAF) is 31%-64% and 4%-32%, respectively. NOAF is independently associated with adverse events such as stroke, death, and increased length of hospital stay. Increasing the knowledge of predisposing factors, optimal postprocedural monitoring, and prophylactic antiarrhythmic and antithrombotic therapy...

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

International Nuclear Information System (INIS)

Wilms, Guido E.; Demaerel, Philippe; Keyzer, Frederik de; Willems, Endry

2012-01-01

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.)

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.)

Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

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Emma A Webb

Full Text Available OBJECTIVES: To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. PATIENTS AND METHODS: Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex was performed. RESULTS: Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (p<0.05 than controls (4 had scores in the clinically significant range. Ventral cingulum, corpus callosum and optic radiation fractional anisotropy were significantly reduced in children with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, p<0.02, r = -0.46, p<0.049 respectively. There were no significant correlations between left ventral cingulum, corpus callosum or optic radiation fractional anisotropy and behavioral scores. CONCLUSIONS: Our findings suggest that children with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic

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

NARCIS (Netherlands)

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

2004-01-01

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

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

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Parikhita Hazarika

2017-08-01

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

A bovine aortic arch in humans

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María Elena Arnáiz-García

2014-05-01

Full Text Available We describe a curious congenital variation of human aortic arch (AA branching pattern termed the “bovine aortic arch”. Rather than arising directly from the AA as a separate branch as occurs in the most common AA branching pattern, the left common carotid artery moves to the right and merges from the brachiocephalic trunk. It is the normal AA branching pattern presented in a number of animals (canines, felines or Macaque monkeys but it has nothing to do with anatomy of AA in ruminant animals, including cattle and buffalo. That is why it is one of the most widely misnomers used in medical literature whose origin is nowadays unknown.

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

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

1991-01-01

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

Acute aortic dissection: be aware of misdiagnosis

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Asteri Theodora

2009-02-01

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

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

International Nuclear Information System (INIS)

Chalard, Francois; Ferey, Solene; Kalifa, Gabriel; Teinturier, Cecile

2005-01-01

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.)

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

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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.)

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

Science.gov (United States)

Chalard, François; Ferey, Solène; Teinturier, Cécile; Kalifa, Gabriel

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.

Dissection of Retroesophageal Aortic Diverticulum and Descending Aorta in a Patient with Right Aortic Arch: Magnetic Resonance Demonstration

International Nuclear Information System (INIS)

Ko, S.-F.; Ng, S.-H.; Fu, Morgan; Lo, P.-H.; Cheng, Y.-F.; Lee, T.-Y.

1996-01-01

An acute aortic dissection involved the retroesophageal aortic diverticulum (RAD) and descending thoracic aorta in a patient with right aortic arch. The RAD, which was separated into false and true lumens by an intimal flap-the classic diagnostic sign of aortic dissection-was overlooked on transesophageal echocardiography and computed tomography but was clearly depicted on magnetic resonance imaging (MRI). It was found that MRI can delineate the anatomy of a congenital arch anomaly complicated by great vessels disease

THE IMPACT OF DIAGNOSTIC DISCREPANCIES IN AORTIC DISSECTION MANAGEMENT

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Abdalla Hassan

2016-01-01

Full Text Available Aortic dissection is uncommon with high mortality rate if untreated. We report a challenging case of long segment aortic dissection in which the dissection type was very difficult to identify due to limitations of the available imaging studies. 66-year-old male presented to us with 3 days history of chest pain and difficulty breathing. He is known to be hypertensive. In the emergency room, patient has systolic blood pressure >190. Chest X-ray showed widening of mediastinum. CT angiography of chest and abdomen showed an acute dissection of the thoracic aorta extending from the mid ascending aorta to the infra-renal aorta suggestive of Stanford type A aortic dissection. Transthoracic and Trans-esophageal echocardiography revealed a partially calcified intimal flap in the distal portion of the arch and in the descending thoracic aorta suggestive of Stanford type B aortic dissection. Medical treatment started, and repeated CT angiography was obtained and it confirmed type B aortic dissection. One week after discharge, patient was readmitted with severe neck pain and difficulty breathing. CT chest without contrast showed grossly stable appearance of type A dissection consistent with the first CT angiography. Cardiothoracic surgery immediately reevaluated the situation and recommended surgical intervention.

Expanding indications for valve-sparing aortic root reconstruction: early and midterm results.

Science.gov (United States)

Valo, Johanna; Jokinen, Janne J; Kaarne, Markku; Ihlberg, Leo

2013-02-01

Valve-sparing aortic root reconstruction (VSRR) is an accepted method to treat patients with aortic root dilation. The role of the VSRR is less well defined for patients with bicuspid aortic valve, severe aortic valve insufficiency, congenital heart defects, and type A aortic dissection. We studied the clinical outcome of patients who underwent VSRR for expanded indications. Seventy-eight patients underwent VSRR between the 2005 and 2012. Seventy-two patients (92%) underwent reimplantation and 6 patients (8%) were operated on with the remodeling technique. The mean age was 51 ± 12 years (range 24 to 73). For 71 patients (91%), the operation was elective, and for 7 (9%; all with type A aortic dissection), on an emergency basis. Preoperatively, the degree of aortic insufficiency was graded as 2+ or greater for 27 patients (35%). Connective tissue disorder (Marfan or Loeys-Dietz), bicuspid aortic valve, or congenital heart disease was present in 15 (19%), 15 (19%), and 7 patients (9%), respectively. Concomitant aortic valve leaflet repair was performed for 39 patients (50%). The mean follow-up time was 2.4 ± 1.7 years (range, 0.1 to 7.0). Thirty-day mortality was zero. The rate of postoperative complications was low: stroke 3%, renal failure 3%, prosthesis infection 1%, and low cardiac output syndrome 1%. Survival was 100% at 1 year and 97% at 5 years. Freedom from recurrent aortic valve insufficiency (≥2+) during the follow-up was 94%. The midterm results of VSRR in terms of survival, freedom from recurrent aortic valve insufficiency, and the need for reoperation are excellent, even for high-risk patients. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Endoluminal treatment of aortic dissection

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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.)

Endoluminal treatment of aortic dissection

International Nuclear Information System (INIS)

Chavan, Ajay; Lotz, Joachim; Galanski, Michael; Oelert, Frank; Haverich, Axel; Karck, Matthias

2003-01-01

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.)

Imaging techniques in aortic valve and root surgery

NARCIS (Netherlands)

Regeer, M.V.

2017-01-01

Aortic valve sparing surgery for aortic regurgitation and/or aortopathy serves as an alternative to aortic valve and root replacement. One of the advantages of aortic valve sparing surgery over conventional replacement is that there is no need for life-long anticoagulation, which is particularly

The influence of Marfans and bicuspid valves on outcomes following aortic valve reimplantation.

Science.gov (United States)

Martín, Carlos E; García Montero, Carlos; Serrano, Santiago-Fiz; González, Ana; Mingo, Susana; Moñivas, Vanessa; Centeno, Jorge; Forteza, Alberto

2017-10-01

We analyzed our early and midterm results with aortic valve reimplantation surgery to determine the influence of Marfan syndrome and bicuspid valves on outcomes with this technique. Between March 2004 and December 2015, 267 patients underwent aortic valve reimplantation operations. The mean diameter of the sinuses of Valsalva was 50 ± 3 mm and moderate/severe aortic regurgitation was present in 34.4% of these patients. A bicuspid aortic valve was present in 21% and 40% had Marfan syndrome. Overall 30-day mortality was 0.37% (1/267). Mean follow-up was 59.7 ± 38.7 months. Overall survival at 1, 3, and 5 years was 98 ± 8%, 98 ± 1%, and 94 ± 2%, respectively. Freedom from reoperation and aortic regurgitation >II was 99 ± 5%, 98 ± 8%, 96.7 ± 8%, and 99 ± 6%, 98 ± 1%, 98 ± 1%, respectively at 1, 3, and 5 years follow-up, with no differences between Marfan and bicuspid aortic valve groups. (p = 0.94 and p = 0.96, respectively). No endocarditis or thromboembolic complications were documented, and 93.6% of the patients did not receive any anticoagulation therapy. The reimplantation technique for aortic root aneurysms is associated with excellent clinical and functional outcomes at short and mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Endocarditis is not an Independent Predictor of Blood Transfusion in Aortic Valve Replacement Patients With Severe Aortic Regurgitation.

Science.gov (United States)

Dahn, Hannah; Buth, Karen; Legare, Jean-Francois; Mingo, Heather; Kent, Blaine; Whynot, Sara; Scheffler, Matthias

2016-06-01

This study sought to evaluate if the presence of endocarditis was independently associated with increased perioperative blood transfusion in patients undergoing aortic valve replacements (AVR) with aortic regurgitation. This was a retrospective study. Large Canadian tertiary care hospital. Six hundred sixty-two consecutive patients with aortic regurgitation score of 3 or higher undergoing AVR from 1995 to 2012. No interventions were performed in this retrospective study. After REB approval, data were obtained from a center-specific database. Univariate analysis was performed to identify variables that may be associated with transfusion of any allogeneic blood product perioperatively. A multivariate logistic regression was generated to identify independent predictors of perioperative transfusion. Unadjusted transfusion rates in patients with no endocarditis and with endocarditis were 32% and 70% (p70, urgent/emergent surgery, BMIEndocarditis was not an independent predictor of transfusion (OR = 0.748; 95% CI = 0.35-1.601). In patients undergoing AVR, unadjusted perioperative transfusion rates were higher when endocarditis was present. However, after adjustment, aortic valve endocarditis was not independently associated with blood transfusion. The authors' observation could be explained by the higher prevalence of many independent predictors of transfusion, such as comorbidities or more complex surgery, within the endocarditis group. Thus, AV endocarditis, in the absence of other risk factors, was not associated with increased perioperative transfusion risk. Copyright © 2016 Elsevier Inc. All rights reserved.

Valve-sparing aortic root replacement†.

Science.gov (United States)

Koolbergen, David R; Manshanden, Johan S J; Bouma, Berto J; Blom, Nico A; Mulder, Barbara J M; de Mol, Bas A J M; Hazekamp, Mark G

2015-02-01

To evaluate our results of valve-sparing aortic root replacement and associated (multiple) valve repair. From September 2003 to September 2013, 97 patients had valve-sparing aortic root replacement procedures. Patient records and preoperative, postoperative and recent echocardiograms were reviewed. Median age was 40.3 (range: 13.4-68.6) years and 67 (69.1%) were male. Seven (7.2%) patients were younger than 18 years, the youngest being 13.4 years. Fifty-four (55.7%) had Marfan syndrome, 2 (2.1%) other fibrous tissue diseases, 15 (15.5%) bicuspid aortic valve and 3 (3.1%) had earlier Fallot repair. The reimplantation technique was used in all, with a straight vascular prosthesis in 11 (26-34 mm) and the Valsalva prosthesis in 86 (26-32 mm). Concomitant aortic valve repair was performed in 43 (44.3%), mitral valve repair in 10 (10.3%), tricuspid valve repair in 5 (5.2%) and aortic arch replacement in 3 (3.1%). Mean follow-up was 4.2 ± 2.4 years. Follow-up was complete in all. One 14-year old patient died 1.3 years post-surgery presumably of ventricular arrhythmia. One patient underwent reoperation for aneurysm of the proximal right coronary artery after 4.9 years and 4 patients required aortic valve replacement, 3 of which because of endocarditis after 0.1, 0.8 and 1.3 years and 1 because of cusp prolapse after 3.8 years. No thrombo-embolic complications occurred. Mortality, root reoperation and aortic regurgitation were absent in 88.0 ± 0.5% at 5-year follow-up. Results of valve-sparing root replacement are good, even in association with a high incidence of concomitant valve repair. Valve-sparing aortic root replacement can be performed at a very young age as long as an adult size prosthesis can be implanted. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Systematic review of the outcome of aortic valve replacement in patients with aortic stenosis

NARCIS (Netherlands)

Sharma, Umesh C.; Barenbrug, Paul; Pokharel, Saraswati; Dassen, Willem R. M.; Pinto, Yigal M.; Maessen, Jos G.

2004-01-01

BACKGROUND: After the establishment of aortic valve replacement procedure for aortic stenosis, there are heterogeneous studies and varying reports on outcome. An analysis that compares individual studies to summarize the overall effect is still lacking. This study systematically analyzes the change

Imaging in aortic dissection

International Nuclear Information System (INIS)

Yu-Qing Liu, M.D.

1995-01-01

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.)

Imaging in aortic dissection

Energy Technology Data Exchange (ETDEWEB)

Yu-Qing Liu, M D [Chinese Academy of Medical Sciences, Beijing, BJ (China). Dept. of Radiology, Fu Wai Hospital and Cardiovascular Inst.

1996-12-31

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.).

Evaluation of the aortic anulus

International Nuclear Information System (INIS)

Link, K.M.; Margosian, P.

1991-01-01

This paper evaluates the efficacy of echocardiography and MR imaging for measuring the aortic anulus in patients who are candidates for aortic valve replacement. The MR imaging study was performed on a 1.5-T system, and the results were compared with echocardiography results obtained with use of a Toshiba system with a 2.5-mHz transducer. The aortic valve anulus was evaluated in the coronal, long-axis, and short-axis views with the MR imaging technique and in the right parasternal suprasternal, and apical projections with the echocardiographic technique. Twenty-four patients studied with MR imaging and echocardiography went on to have aortic valve replacement. When compared with surgical results, MR imaging had an r value of .95 while echocardiography had an r value of .70

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

Science.gov (United States)

Kallenbach, K; Schwill, S; Karck, M

2011-09-01

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

Relation of thoracic aortic and aortic valve calcium to coronary artery calcium and risk assessment.

Science.gov (United States)

Wong, Nathan D; Sciammarella, Maria; Arad, Yadon; Miranda-Peats, Romalisa; Polk, Donna; Hachamovich, Rory; Friedman, John; Hayes, Sean; Daniell, Anthony; Berman, Daniel S

2003-10-15

Aortic calcium, aortic valve calcium (AVC), and coronary artery calcium (CAC) have been associated with cardiovascular event risk. We examined the prevalence of thoracic aortic calcium (TAC) and AVC in relation to the presence and extent of CAC, cardiovascular risk factors, and estimated risk of coronary heart disease (CHD). In 2,740 persons without known CHD aged 20 to 79 years, CAC was assessed by electron beam- or multidetector-computed tomography. We determined the prevalence of TAC and AVC in relation to CAC, CHD risk factors, and predicted 10-year risk of CHD. A close correspondence of TAC and AVC was observed with CAC. TAC and AVC increased with age; by the eighth decade of life, the prevalence of TAC was similar to that of CAC (>80%), and 36% of men and 24% of women had AVC. Age, male gender, and low-density lipoprotein cholesterol were directly related to the likelihood of CAC, TAC, and AVC; higher diastolic blood pressure and cigarette smoking additionally predicted CAC. Body mass index and higher systolic and lower diastolic blood pressures were also related to TAC, and higher body mass index and lower diastolic blood pressure were related to AVC. Calculated risk of CHD increased with the presence of AVC and TAC across levels of CAC. TAC and AVC provided incremental value over CAC in association with the 10-year calculated risk of CHD. If longitudinal studies show an incremental value of aortic and aortic valve calcium over that of CAC for prediction of cardiovascular events, future guidelines for risk assessment incorporating CAC assessment may additionally incorporate the measurement of aortic and/or aortic valve calcium.

Recessive Mutations in SLC38A8 Cause Foveal Hypoplasia and Optic Nerve Misrouting without Albinism

NARCIS (Netherlands)

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

2013-01-01

Foveal hypoplasia and optic nerve misrouting are developmental defects of the visual pathway and only co-occur in connection with albinism; to date, they have only been associated with defects in the melanin-biosynthesis pathway. Here, we report that these defects can occur independently of albinism

Long-term outcomes after immediate aortic repair for acute type A aortic dissection complicated by coma.

Science.gov (United States)

Tsukube, Takuro; Haraguchi, Tomonori; Okada, Yasushi; Matsukawa, Ritsu; Kozawa, Shuichi; Ogawa, Kyoichi; Okita, Yutaka

2014-09-01

The management of acute type A aortic dissection complicated by coma remains controversial. We previously reported an excellent rate of recovery of consciousness provided aortic repair was performed within 5 hours of the onset of symptoms. This study evaluates the early and long-term outcomes using this approach. Between August 2003 and July 2013, of the 241 patients with acute type A aortic dissection brought to the Japanese Red Cross Kobe Hospital and Hyogo Emergency Medical Center, 30 (12.4%) presented with coma; Glasgow Coma Scale was less than 11 on arrival. Surgery was performed in 186 patients, including 27 (14.5%) who were comatose. Twenty-four comatose patients underwent successful aortic repair immediately (immediate group). Their mean age was 71.0 ± 11.1 years, Glasgow Coma Scale was 6.5 ± 2.4, and prevalence of carotid dissection was 79%. For brain protection, deep hypothermia with antegrade cerebral perfusion was used, and postoperative induced hypothermia was performed. Neurologic evaluations were performed using the Glasgow Coma Scale, National Institutes of Health Stroke Scale, and modified Rankin Scale. In the immediate group, the time from the onset of symptoms to arrival in the operating theater was 222 ± 86 minutes. Hospital mortality was 12.5%. Full recovery of consciousness was achieved in 79% of patients in up to 30 days. Postoperative Glasgow Coma Scale and National Institutes of Health Stroke Scale improved significantly when compared with the preoperative score (P coma were satisfactory. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

Schizencephaly with optic nerve hypoplasia simulating septo-optic dysplasia and other syndromes

International Nuclear Information System (INIS)

Osborn, R.E.; Byrd, S.E.

1988-01-01

Three patients with fused-lip schizencephaly with optic nerve hypoplasia were evaluated radiographically. The computed tomographic and magnetic resonance findings demonstrated unilateral or bilateral gray matter lined clefts in the cerebral hemisphere which allowed differentiation from the clinical diagnosis of septo-optic dysplasia in each case. Schizencephaly has also been mistakenly labelled as part of a syndrome known as the syndrome of absence of the septum pellucidum with porencephalies and as heterotopias with absence of the septum pellucidum. 15 refs.; 3 figs

New predictor of aortic enlargement in uncomplicated type B aortic dissection based on elliptic Fourier analysis.

Science.gov (United States)

Sato, Hiroshi; Ito, Toshiro; Kuroda, Yosuke; Uchiyama, Hiroki; Watanabe, Toshitaka; Yasuda, Naomi; Nakazawa, Junji; Harada, Ryo; Kawaharada, Nobuyoshi

2017-12-01

This study aimed to re-examine the conventional predictive factors for dissected aortic enlargement, such as the aortic and false lumen diameter and to consider whether the morphological elements of the dissected aorta could be predictors by quantifying the 'shape' of the true lumen based on elliptic Fourier analysis. A total of 80 patients with uncomplicated type B aortic dissection were included. The patients were divided into 'Enlargement group' and 'No Change group.' Between the 2 groups, the mean systolic blood pressure during follow-up, aortic and false lumen maximum diameters, and analysed morphological data were compared using each statistical method. The maximum aortic and false lumen diameters were significantly larger in the Enlargement group than in the No Change group (39.3 vs 35.9 mm; P = 0.0058) (23.5 vs 18.2 mm; P = 0.000095). The principal component 1, which is the data calculated by elliptic Fourier analysis, was significantly lower in the Enlargement group than in the No Change group (0.020 vs - 0.072; P = 0.000049). The mean systolic blood pressure ≥130 mmHg, aortic diameter, false lumen diameter and principal component 1 were included in the Cox proportional hazard model as covariates to determine the significant predictive variable. Principal component 1 demonstrated the only significance with aortic enlargement on multivariate analysis (odds ratio = 0.32; P = 0.048). The analysed and calculated morphological data of the shape of the true lumen can be more effective predictive factors of aortic enlargement of type B dissection than the conventional factors. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Interobserver variability of CT angiography for evaluation of aortic annulus dimensions prior to transcatheter aortic valve implantation (TAVI)

Energy Technology Data Exchange (ETDEWEB)

Schmidkonz, C., E-mail: christian.schmidkonz@gmail.com [Department of Internal Medicine 2 (Cardiology), University of Erlangen, Ulmenweg 18, D-91054 Erlangen (Germany); Marwan, M.; Klinghammer, L.; Mitschke, M.; Schuhbaeck, A.; Arnold, M. [Department of Internal Medicine 2 (Cardiology), University of Erlangen, Ulmenweg 18, D-91054 Erlangen (Germany); Lell, M. [Radiological Institute, University of Erlangen, Maximiliansplatz 1, D-91054 Erlangen (Germany); Achenbach, S.; Pflederer, T. [Department of Internal Medicine 2 (Cardiology), University of Erlangen, Ulmenweg 18, D-91054 Erlangen (Germany)

2014-09-15

Highlights: • Cardiac CT provides highly reproducible measurements of aortic annulus and root dimensions prior to TAVI. • The perimeter-derived aortic annulus diameter shows the lowest interobserver variability. • If all three CT sizing methods are considered and stated as a “consensus result”, mismatches in prosthesis size selection can be further reduced. - Abstract: Objective: Assessment of aortic annulus dimensions prior to transcatheter aortic valve implantation (TAVI) is crucial for accurate prosthesis sizing in order to avoid prosthesis–annulus-mismatch possibly resulting in complications like valve dislodgement, paravalvular regurgitation or annulus rupture. Contrast-enhanced multidetector computed tomography allows 3-dimensional assessment of aortic annulus dimensions. Only limited data exist about its interobserver variability. Methods: In 100 consecutive patients with symptomatic severe aortic stenosis (51 male, BMI 27 ± 5 kg/m{sup 2}, age 81 ± 7 years, heart rate 72 ± 15 bpm, Logistic Euroscore 31 ± 14%, STS-Score 7 ± 4%), pre-interventional aortic annulus assessment was performed by dual source computed tomography (collimation 2 × 128 × 0.6 mm, high pitch spiral data acquisition mode, 40–60 ml contrast agents, radiation dose 3.5 ± 0.9 mSv). The following aortic annulus characteristics were determined by three independent observers: aortic annulus maximum, minimum and mean diameters (D{sub max}, D{sub min}, D{sub mean}), eccentricity index (EI), effective aortic annulus diameter according to its circumference (D{sub circ}), effective aortic annulus diameter according to its area (D{sub area}), distance from the aortic annulus plane to the left (LCA) and right coronary artery (RCA) ostia, maximum (D{sub max}AR) and minimum aortic root diameter (D{sub min}AR), maximum (D{sub max}STJ) and minimum diameter of the sinotubular junction (D{sub min}STJ). Subsequently, interobserver variabilities were assessed. Results: Correlation between

Traumatic aortic injury score (TRAINS): an easy and simple score for early detection of traumatic aortic injuries in major trauma patients with associated blunt chest trauma.

Science.gov (United States)

Mosquera, Victor X; Marini, Milagros; Muñiz, Javier; Asorey-Veiga, Vanesa; Adrio-Nazar, Belen; Boix, Ricardo; Lopez-Perez, José M; Pradas-Montilla, Gonzalo; Cuenca, José J

2012-09-01

To develop a risk score based on physical examination and chest X-ray findings to rapidly identify major trauma patients at risk of acute traumatic aortic injury (ATAI). A multicenter retrospective study was conducted with 640 major trauma patients with associated blunt chest trauma classified into ATAI (aortic injury) and NATAI (no aortic injury) groups. The score data set included 76 consecutive ATAI and 304 NATAI patients from a single center, whereas the validation data set included 52 consecutive ATAI and 208 NATAI patients from three independent institutions. Bivariate analysis identified variables potentially influencing the presentation of aortic injury. Confirmed variables by logistic regression were assigned a score according to their corresponding beta coefficient which was rounded to the closest integer value (1-4). Predictors of aortic injury included widened mediastinum, hypotension less than 90 mmHg, long bone fracture, pulmonary contusion, left scapula fracture, hemothorax, and pelvic fracture. Area under receiver operating characteristic curve was 0.96. In the score data set, sensitivity was 93.42 %, specificity 85.85 %, Youden's index 0.79, positive likelihood ratio 6.60, and negative likelihood ratio 0.08. In the validation data set, sensitivity was 92.31 % and specificity 85.1 %. Given the relative infrequency of traumatic aortic injury, which often leads to missed or delayed diagnosis, application of our score has the potential to draw necessary clinical attention to the possibility of aortic injury, thus providing the chance of a prompt specific diagnostic and therapeutic management.

Prophylactic irradiation of para-aortic lymph nodes in carcinoma of the uterine cervix

International Nuclear Information System (INIS)

Chatani, M.; Matayoshi, Y.; Masaki, N.; Narumi, Y.; Teshima, T.; Inoue, T.

1995-01-01

For assessment of the advantages and side effects of para-aortic lymph nodes irradiation under the evaluation by computer tomography, a prospective randomized study was started in 1986. The results for survival, local control and late complications are presented in the following. From November 1986 to October 1990, 93 patients with cervical carcinoma were randomly allocated for treatment with either pelvic irradiation (pelvic group) or pelvic plus para-aortic lymph nodes irradiation (para-aortic group). Thirty-six patients underwent external irradiation and intracavitary therapy (RT arm) and 57 patients, extended radical hysterectomy and external irradiation (OP-RT arm). Para-aortic lymph nodes irradiation delivered 45 Gy in 1.8 Gy per day for 5 days per week through anterior-posterior fields. The 3-year cause specific survival rates were para-aortic group: 57% and pelvic group: 89% in RT arm group, and para-aortic group: 70% and pelvic group: 86% in OP-RT arm group. Differences for the 2 groups in each treatment arm were not significant. In pelvic failure, para-aortic lymph nodes metastases and distant metastases showed no statistically significant differences for the 2 groups in each treatment arm. In the para-aortic group, complications were more frequent than in the pelvic group (13/45 vs. 2/48, p [de

Renal Infarction from Type B Aortic Dissection

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Marit Tweet

2017-07-01

Full Text Available History of present illness: A 69-year-old-female with a medical history of hypertension presented to the emergency department (ED complaining of sudden onset of lower back and abdominal pain. Eight hours prior to presentation she reported turning to grab something on another counter and felt a sudden onset sharp pain in her lower back. She began to notice ill-defined abdominal pain, although she denied any shortness of breath, chest pain, numbness, weakness, or urologic symptoms. Her vital signs on presentation to the ED were temperature 36.5˚C, blood pressure 143/88, heart rate 84, respiratory rate 14, and oxygen saturation 93% on room air. On arrival, the patient was in significant discomfort and was rolling on the bed due to pain. Although she appeared to be in moderate distress she had minimal, if any, tenderness on abdominal exam, and only mild left lower back tenderness to palpation. This disconnect between the patient’s apparent discomfort and the lack of physical exam findings prompted the ordering of a computed tomography angiography (CTA of the abdomen/pelvis with greatest suspicion for abdominal aortic aneurysm. Significant findings: Initial abdominal images demonstrated a dissection flap; therefore, a CTA of the chest was also obtained. These images revealed a Stanford type B aortic dissection beginning just distal to the left subclavian artery and extending to the origin of the inferior mesenteric artery. The right renal artery arose from the true lumen of the dissection while the left renal artery arose from the false lumen. This case is interesting as imaging shows the lack of perfusion to the left kidney, residing in the retroperitoneum, which correlates with her non-descript abdominal and left flank pain. Discussion: Aortic dissection is defined as a tear within the wall of the aorta that allows blood to track between intima and media layers. A dissection is classified as Stanford Type A if it involves any portion of the

Why aortic elasticity differs among classical and non-classical mitral valve prolapsed?

Science.gov (United States)

Unlu, Murat; Demirkol, Sait; Aparci, Mustafa; Arslan, Zekeriya; Balta, Sevket; Dogan, Umuttan; Kilicarslan, Baris; Ozeke, Ozcan; Celik, Turgay; Iyisoy, Atila

2014-01-01

Mitral valve prolapse (MVP) is the most common valvular heart disease and characterized by the displacement of an abnormally thickened mitral valve leaflet into the left atrium during systole. There are two types of MVP, broadly classified as classic (thickness ≥5 mm) and non-classic (thickness elastic properties of the aorta in young male patients with classical and non-classical MVP. In the present study, 63 young adult males (mean age: 22.7 ± 4.2) were included. Patients were divided into classic MVP (n = 27) and non-classic MVP (n = 36) groups. Aortic strain, aortic distensibility and aortic stiffness index were calculated by using aortic diameters obtained by echocardiography and blood pressures measured by sphygmomanometer. There was no significant difference between the groups in terms of age, body mass index, left ventricular mass and ejection fraction. When comparing the MVP group it was found that aortic strain and aortic distensibility were increased (p = 0.0027, p = 0.016, respectively) whereas the aortic stiffness index was decreased (p = 0.06) in the classical MVP group. We concluded that the elastic properties of the aorta is increased in patients with classic MVP. Further large scale studies should be performed to understand of morphological and physiological properties of the aorta in patients with MVP.

Elevation of Matrix Metalloproteinases in Different Areas of Ascending Aortic Aneurysms in Patients with Bicuspid and Tricuspid Aortic Valves

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Salah A. Mohamed

2012-01-01

Full Text Available Our aim is to investigate the elevation of matrix proteins in tissues obtained from distal, above the sinotubular junction (proximal, concave, and convex sites of aneurysms in the ascending aorta using a simultaneous multiplex protein detection system. Tissues were collected from 41 patients with ascending aortic aneurysms. A total of 31 patients had a bicuspid aortic valve (BAV, whereas 10 had a tricuspid aortic valve (TAV. Concave and convex aortic site samples were collected from all patients, whereas proximal and distal convexity samples were obtained from 19 patients with BAV and 7 patients with TAV. Simultaneous detection of matrix metalloproteinases (MMPs and their inhibitors (TIMPs was performed at each of the four aortic sites. MMP-2 levels were higher in the concave aortic sites than in the convex aortic sites. In contrast, MMP-8 levels were higher in the convex sites than in the concave sites, as were MMP-9 levels. In both BAV and TAV patients, TIMP-3 levels were higher in the concave sites than in the convex sites. However, TIMP-2 and TIMP-4 levels were significantly elevated in the sinotubular proximal aorta of BAV patients. Simultaneous detection of MMPs and TIMPs revealed different levels at different aortic sites in the same patient.

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

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

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...

Severe aortic valve stenosis and nosebleed

NARCIS (Netherlands)

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

2007-01-01

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

 Ischemic Stroke Secondary to Aortic Dissection Following Rifle Butt Recoil Chest Injury: A Case Report

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John Valiath

2011-11-01

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

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

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Sarah Morrissey, MD, MA, FRCSC

2017-09-01

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

Simultaneous rota-stenting and transcatheter aortic valve implantation for patients with heavily calcified coronary stenosis and aortic stenosis

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Yung-Tsai Lee

2016-09-01

Full Text Available Given that coronary artery disease (CAD in octogenarians undergoing transcatheter aortic valve implantation (TAVI often presents with more complex lesions and extensive calcification, rotational atherectomy (RA may be needed in some cases before stenting. However, data regarding the feasibility and safety of simultaneous RA during TAVI using the Medtronic CoreValve (MCV; Medtronic, Minneapolis, MN, USA system are lacking. Three out of 107 (2.8% patients (2 females, average age 85.6 years, mean aortic valve area 0.5 cm2, mean left ventricular ejection fraction 39%, mean Logistic EuroScore 70%, with complex, heavily calcified coronary stenosis, and severe valvular aortic stenosis (AS were treated with TAVI and RA due to high surgical risk. After balloon valvuloplasty, all coronary lesions were successfully treated with RA and stenting, immediately followed by transfemoral TAVI with a self-expandable MCV. Our data suggested that in the very elderly patients with severe and heavily calcified CAD and AS who were turned down for cardiac surgery, RA and stenting followed by TAVI may be performed successfully in a combined, single-stage procedure.

Contemporary management of symptomatic primary aortic mural thrombus.

Science.gov (United States)

Verma, Himanshu; Meda, Narendranadh; Vora, Simit; George, Robbie K; Tripathi, Ramesh K

2014-12-01

Primary aortic mural thrombus (PAMT) is an uncommon condition but an important source of noncardiogenic emboli with a difficult diagnosis and a high rate of complications, including high mortality. We report our experience of thromboembolic disease from PAMT and review its contemporary management. Retrospective analysis of prospectively collected data of all patients who presented with acute occlusion of a limb or visceral vessels between January 2011 and September 2013 was performed. A total of 88 patients presented with acute occlusion of the extremities or visceral arteries. All underwent extensive evaluation for the possible source of the embolism. Of these 88 patients, 19 patients (mean age, 41.2 years; male:female ratio, 1:2.1) were found to have aortic mural thrombus as the source of distal embolism. Thrombus was located in the thoracic aorta in 10 patients, in the perivisceral aorta in three patients, and in the infrarenal aorta in six patients. Thrombus in the thoracic aorta was treated with stent grafts in four patients, bare metal stents in three patients, and anticoagulation alone in two patients. In the suprarenal abdominal aorta, all three patients underwent trapdoor aortic thrombectomy. Infrarenal aortic thrombus was managed by aortobifemoral embolectomy in two patients, aortic stenting in two patients, surgical thrombectomy in one patient, and anticoagulation alone in one patient. Successful treatment, defined as freedom from further embolic events or recurrence of thrombus, was achieved in 14 of 19 patients (76.4%) with a mean follow-up period of 16.2 months (range, 2-28 months). There were four (21%) thrombus-related deaths, all due to primary thromboembolic insults. One patient needed a below-knee amputation because of a recurrent thrombotic episode. Symptomatic PAMT is an uncommon but important source of noncardiogenic embolus. It appears to occur more frequently in young women. Endovascular coverage of the aortic thrombus, when feasible

Cost-effectiveness of transcatheter aortic valve replacement compared with surgical aortic valve replacement in high-risk patients with severe aortic stenosis: results of the PARTNER (Placement of Aortic Transcatheter Valves) trial (Cohort A).

Science.gov (United States)

Reynolds, Matthew R; Magnuson, Elizabeth A; Lei, Yang; Wang, Kaijun; Vilain, Katherine; Li, Haiyan; Walczak, Joshua; Pinto, Duane S; Thourani, Vinod H; Svensson, Lars G; Mack, Michael J; Miller, D Craig; Satler, Lowell E; Bavaria, Joseph; Smith, Craig R; Leon, Martin B; Cohen, David J

2012-12-25

The aim of this study was to evaluate the cost-effectiveness of transcatheter aortic valve replacement (TAVR) compared with surgical aortic valve replacement (AVR) for patients with severe aortic stenosis and high surgical risk. TAVR is an alternative to AVR for patients with severe aortic stenosis and high surgical risk. We performed a formal economic analysis based on cost, quality of life, and survival data collected in the PARTNER A (Placement of Aortic Transcatheter Valves) trial in which patients with severe aortic stenosis and high surgical risk were randomized to TAVR or AVR. Cumulative 12-month costs (assessed from a U.S. societal perspective) and quality-adjusted life-years (QALYs) were compared separately for the transfemoral (TF) and transapical (TA) cohorts. Although 12-month costs and QALYs were similar for TAVR and AVR in the overall population, there were important differences when results were stratified by access site. In the TF cohort, total 12-month costs were slightly lower with TAVR and QALYs were slightly higher such that TF-TAVR was economically dominant compared with AVR in the base case and economically attractive (incremental cost-effectiveness ratio economically dominated by AVR in the base case and economically attractive in only 7.1% of replicates. In the PARTNER trial, TAVR was an economically attractive strategy compared with AVR for patients suitable for TF access. Future studies are necessary to determine whether improved experience and outcomes with TA-TAVR can improve its cost-effectiveness relative to AVR. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Blood Pressure and Arterial Load After Transcatheter Aortic Valve Replacement for Aortic Stenosis.

Science.gov (United States)

Lindman, Brian R; Otto, Catherine M; Douglas, Pamela S; Hahn, Rebecca T; Elmariah, Sammy; Weissman, Neil J; Stewart, William J; Ayele, Girma M; Zhang, Feifan; Zajarias, Alan; Maniar, Hersh S; Jilaihawi, Hasan; Blackstone, Eugene; Chinnakondepalli, Khaja M; Tuzcu, E Murat; Leon, Martin B; Pibarot, Philippe

2017-07-01

After aortic valve replacement, left ventricular afterload is often characterized by the residual valve obstruction. Our objective was to determine whether higher systemic arterial afterload-as reflected in blood pressure, pulsatile and resistive load-is associated with adverse clinical outcomes after transcatheter aortic valve replacement (TAVR). Total, pulsatile, and resistive arterial load were measured in 2141 patients with severe aortic stenosis treated with TAVR in the PARTNER I trial (Placement of Aortic Transcatheter Valve) who had systolic blood pressure (SBP) and an echocardiogram obtained 30 days after TAVR. The primary end point was 30-day to 1-year all-cause mortality. Lower SBP at 30 days after TAVR was associated with higher mortality (20.0% for SBP 100-129 mm Hg versus 12.0% for SBP 130-170 mm Hg; P <0.001). This association remained significant after adjustment, was consistent across subgroups, and confirmed in sensitivity analyses. In adjusted models that included SBP, higher total and pulsatile arterial load were associated with increased mortality ( P <0.001 for all), but resistive load was not. Patients with low 30-day SBP and high pulsatile load had a 3-fold higher mortality than those with high 30-day SBP and low pulsatile load (26.1% versus 8.1%; hazard ratio, 3.62; 95% confidence interval, 2.36-5.55). Even after relief of valve obstruction in patients with aortic stenosis, there is an independent association between post-TAVR blood pressure, systemic arterial load, and mortality. Blood pressure goals in patients with a history of aortic stenosis may need to be redefined. Increased pulsatile arterial load, rather than blood pressure, may be a target for adjunctive medical therapy to improve outcomes after TAVR. URL: http://www.clinicaltrials.gov. Unique identifier: NCT00530894. © 2017 American Heart Association, Inc.

Aortic stenosis: From diagnosis to optimal treatment

Directory of Open Access Journals (Sweden)

Tavčiovski Dragan

2008-01-01

Full Text Available Aortic stenosis is the most frequent valvular heart disease. Aortic sclerosis is the first characteristic lesion of the cusps, which is considered today as the process similar to atherosclerosis. Progression of the disease is an active process leading to forming of bone matrix and heavily calcified stiff cusps by inflammatory cells and osteopontin. It is a chronic, progressive disease which can remain asymptomatic for a long time even in the presence of severe aortic stenosis. Proper physical examination remains an essential diagnostic tool in aortic stenosis. Recognition of characteristic systolic murmur draws attention and guides further diagnosis in the right direction. Doppler echocardiography is an ideal tool to confirm diagnosis. It is well known that exercise tests help in stratification risk of asymptomatic aortic stenosis. Serial measurements of brain natriuretic peptide during a follow-up period may help to identify the optimal time for surgery. Heart catheterization is mostly restricted to preoperative evaluation of coronary arteries rather than to evaluation of the valve lesion itself. Currently, there is no ideal medical treatment for slowing down the disease progression. The first results about the effect of ACE inhibitors and statins in aortic sclerosis and stenosis are encouraging, but there is still not enough evidence. Onset symptoms based on current ACC/AHA/ESC recommendations are I class indication for aortic valve replacement. Aortic valve can be replaced with a biological or prosthetic valve. There is a possibility of percutaneous aortic valve implantation and transapical operation for patients that are contraindicated for standard cardiac surgery.

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.

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.)

Lipoprotein(a Induces Human Aortic Valve Interstitial Cell Calcification

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Bin Yu, PhD

2017-08-01

Full Text Available Lipoprotein(a, or Lp(a, significantly increased alkaline phosphatase activity, release of phosphate, calcium deposition, hydroxyapatite, cell apoptosis, matrix vesicle formation, and phosphorylation of signal transduction proteins; increased expression of chondro-osteogenic mediators; and decreased SOX9 and matrix Gla protein (p < 0.001. Inhibition of MAPK38 and GSK3β significantly reduced Lp(a-induced calcification of human aortic valve interstitial cells (p < 0.001. There was abundant presence of Lp(a and E06 immunoreactivity in diseased human aortic valves. The present study demonstrates a causal effect for Lp(a in aortic valve calcification and suggests that interfering with the Lp(apathway could provide a novel therapeutic approach in the management of this debilitating disease.

Patient-Reported Esthetic and Functional Outcomes of Primary Total Laparoscopic Intestinal Vaginoplasty in Transgender Women With Penoscrotal Hypoplasia

NARCIS (Netherlands)

Bouman, M.B.; Sluis, W.B. van der; Woudenberg Hamstra, L.E. van; Buncamper, M.E.; Kreukels, B.P.; Meijerink, W.J.H.J.; Mullender, M.G.

2016-01-01

INTRODUCTION: Puberty-suppressing hormonal treatment may result in penoscrotal hypoplasia in transgender women, making standard penile inversion vaginoplasty not feasible. For these patients, intestinal vaginoplasty is a surgical alternative, but knowledge on patient-reported postoperative outcomes

Regional cerebral perfusion for surgical correction of neonatal aortic arch obstruction.

Science.gov (United States)

Zhang, Hui; Cheng, Pei; Hou, Jia; Li, Lei; Liu, Hu; Liu, Ruifang; Ji, Bingyang; Luo, Yi

2009-05-01

One-stage repair of aortic arch obstruction and associated cardiac anomalies is a surgical challenge in infants.The purpose of the present study is to review the current outcome using regional cerebral perfusion (RCP) during a procedure correcting interrupted aortic arch (IAA) and also isolated aortic coarctation (CoA) and CoA combined with hypoplastic aortic arch (CoA-HyAA) in our center. Between January 2007 and July 2008, 24 infant patients with interrupted aortic arch (IAA) (n=3), isolated aortic coarctation (iCoA) (n=9) and aortic coarctation with hypoplastic aortic arch (CoA-HyAA) (n=12) underwent one-stage surgical correction in our hospital. End-to-end anastomosis was employed in 12 infants (IAA n=3 and iCoA n=9); for the other 12 patients with CoA-HyAA, an end-to-end extended anastomosis was used in 8 cases, end-to-side anastomosis in 2 cases, and composite heterologous pericardial patch in 2 cases. RCP with 40 mL/kg/min through the innominate artery during aortic arch reconstruction was employed for all pediatric patients. One single-dose histidine-ketoglutarate-tryptophan (HTK) solution was used for myocardial protection during CPB. Cardiopulmonary bypass time and aortic cross-clamp time were 165.6+/-32.4 min and 81.7+/-30.0 min, respectively. The mean regional cerebral perfusion time was 31.0+/-10.6 min; lowest nasopharyngeal temperature was 19.1+/-1.1 degrees C. Operative mortality rate in both groups was 8.3%. Mean follow-up was 10.5+/-4.8 months. There was no late mortality or postoperative neurologic, renal or hepatic complications. All patients are asymptomatic and are developing normally. One-stage total arch repair using the RCP technique is an excellent method that may minimize neurologic and renal complications. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in these infants.

Aortic reconstruction with bovine pericardial grafts

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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.

Infrarenal Abdominal Aortic Pseudoaneurysm: Is It a Real Emergency?

Science.gov (United States)

Massara, Mafalda; Prunella, Roberto; Gerardi, Pasquale; Lillo, Antonio; De Caridi, Giovanni; Serra, Raffaele; Notarstefano, Stefano; Impedovo, Giovanni

2017-01-01

Abdominal aortic pseudoaneurysm is a rare but life-threatening condition that occurs due to penetrating or blunt trauma. Clinical manifestations are variable, and the time interval from the initial trauma to diagnosis is variable. A prompt diagnosis and an aggressive management approach are required to avoid catastrophic complications. Possible treatment options are open surgical repair, endovascular repair, pseudoanerysmal sac thrombosis induction through direct thrombin injection, and coil embolization. Here, we present the case of a 75-year-old man affected by an infrarenal abdominal aortic pseudoaneurysm presenting with abdominal and lumbar pain for 3 days, who was successfully treated with an endograft. PMID:29515707

Aortic valve replacement

DEFF Research Database (Denmark)

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

2008-01-01

mortality were collected. Group analysis by patient geographic distribution and by annular diameter of the prosthesis utilized was conducted. Patients with a manufacturer's labeled prosthesis size > or = 21 mm were assigned to the 'large' aortic size subset, while those with a prosthesis size ... differences in the distribution of either gender or BSA. In the multivariable model, south European patients were seven times more likely to receive a smaller-sized aortic valve (OR = 6.5, 95% CI = 4.82-8.83, p

Valve-sparing aortic root replacement†

NARCIS (Netherlands)

Koolbergen, David R.; Manshanden, Johan S. J.; Bouma, Berto J.; Blom, Nico A.; Mulder, Barbara J. M.; de Mol, Bas A. J. M.; Hazekamp, Mark G.

2015-01-01

To evaluate our results of valve-sparing aortic root replacement and associated (multiple) valve repair. From September 2003 to September 2013, 97 patients had valve-sparing aortic root replacement procedures. Patient records and preoperative, postoperative and recent echocardiograms were reviewed.

Thoracic aortic aneurysm in a child due to cystic medial necrosis

International Nuclear Information System (INIS)

Kuribayashi, Sachio; Watabe, Tsuneya; Ohtaki, Makoto; Matsuyama, Shoya; Ogawa, Junichi

1983-01-01

The valuable role of computed tomography (CT) was stressed in the diagnosis of thoracic aortic aneurysm in an asymptomatic 12-year-old child. She initially presented mediastinal mass on plain chest film. A saccular thoracic aortic aneurysm was highly suspected from the CT findings, and it was confirmed on angiography. Pathological examination of the aneurysmal wall revealed cystic medial necrosis. (author)

Surgical treatment of infective endocarditis with aortic and tricuspid valve involvement using cryopreserved aortic and mitral valve allografts.

Science.gov (United States)

Ostrovsky, Yury; Spirydonau, Siarhei; Shchatsinka, Mikalai; Shket, Aliaksandr

2015-05-01

Surgical treatment of infective and prosthetic endocarditis using allografts gives good results. Aortic allograft implantation is a common technique, while tricuspid valve replacement with a mitral allograft is very rare. Multiple valve disease in case of infective endocarditis is a surgical challenge as such patients are usually in a grave condition and results of surgical treatment are often unsatisfactory. In this article we describe a clinical case of successful surgical treatment in a patient with active infective endocarditis of aortic and tricuspid valve, complicated by an aortic-right ventricular fistula. The aortic valve and ascending aorta were replaced with a cryopreserved aortic allograft; the tricuspid valve was replaced with a cryopreserved mitral allograft. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Strategy to avoid patient-prosthesis mismatch: aortic root enlargement.

Science.gov (United States)

Srivastava, Dharmendra Kumar; Sanki, Prokash; Bhattacharya, Subhankar; Siddique, Javed Veqar

2014-02-01

The choice of a valve with an effective orifice area matching the body surface area and providing efficient hemodynamics is an important factor affecting mortality and morbidity in patients undergoing aortic valve replacement. Our preventative strategy was to implant a larger prosthetic valve by aortic root enlargement using the Nunez procedure in 17 patients between February 2010 and January 2011. The decision to enlarge the aortic root was taken when the 19-mm sizer could not be negotiated easily through the aortic root, or on the basis of body surface area of the patient or type of prosthesis available. Postoperative reductions in peak and mean pressure gradients across aortic valve of 12.8-16.5 and 10.2-12.6 mm Hg, respectively, were observed. Postoperative effective orifice areas of the aortic valves were 1.1-1.5 cm(2). By upsizing the aortic valve, we were able to eliminate patient-prosthesis mismatch in 5 patients, and reduce severe patient-prosthesis mismatch to moderate in 11. Aortic root enlargement is a safe procedure. Therefore, cardiac surgeons should not be reluctant to enlarge the aortic root with an autologous pericardial patch to permit implantation of an adequate size of aortic valve prosthesis, with minimal additional aortic crossclamp time and no added cost.

A new self-expandable aortic valved stent deployed above native leaflets for aortic insufficiency: an in vitro study.

Science.gov (United States)

Huang, H; Zhou, Y; Shao, J; Cai, J; Mei, Y; Wang, Y

2012-12-01

The aim of this paper was to develop a new self-expandable aortic valved stent following the shape of the sinus of Valsalva, which can be deployed above native leaflets for aortic regurgitation, and study it's effect on coronary artery flow when orthotopic implantation in and above native leaflets. New self-expandable aortic valved stent consist of nitinol stent and bovine pericardium, and was designed following the shape of the sinus of Valsalva, the bovine pericardium was tailed as native leaflet. Thirty-six swine hearts were divided into three equal groups of twelve. In Group A (N.=12), the new self-expandable aortic valved stents deployed in native leaflets. In Group B (N.=12), the new self-expandable aortic valved stents deployed above native leaflets. In Group C (N.=12), the cylinder-like valved stents deployed only in native leaflets. The measurements of each coronary flow rate and endoscopic inspections were repeated post-implantation. In Group A and C, valve implantation in native leaflets resulted in a significant decrease in both left and right coronary flows. In Group B, no significant change in either right or left coronary flow was found after new self-expandable aortic valved stent placement. Endoscopic inspections showed that in group A and C the native leaflets sandwiched between valved stent and aortic wall, whereas, in group B the native leaflets were under the artificial leaflets. Two kinds of stents deployed in native leaflets affect left and right coronary flows significantly. No significant effect was found when the new self-expandable aortic valved stent deployed above native leaflets. This new self-expandable aortic valved stent can be deployed above the native leaflets, which avoids the obstruction of native leaflets on coronary flow.

Malignant Hypertensive Retinopathy in an Infant with Mid-Aortic Occlusion

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Lawrence J. Oh

2016-01-01

Full Text Available Purpose. Case report describing an eight-month-old infant presenting with intermittent esotropia and irritability who was found to have malignant (grade 4 hypertensive retinopathy and mid-aortic syndrome. Methods. Visual acuity was 6/140 in the right eye and not recordable in the left eye. Blood pressure was as high as 230/120 mmHg. Fundoscopy revealed bilateral optic disc swelling, macular stars, and serous retinal detachment in the left eye, findings that are consistent with malignant (grade 4 hypertensive retinopathy. CT abdominal angiogram revealed a severe mid-aortic syndrome with occlusion of the abdominal aorta at T12. Results. The patient was treated with medical management of his hypertension, improving the subretinal exudate. Binocular visual acuity improved to 6/9.5 over 9 months. There was a persistent left relative afferent pupillary defect and moderate left esotropia. Conclusion. This is the first reported case of malignant hypertensive retinopathy in an infant with concomitant mid-aortic occlusion. The authors emphasize the need for an ophthalmological and pediatric examination in a child presenting with intermittent squint and irritability. The esotropia was found to be a false localizing sign of raised intracranial pressure secondary to the severe mid-aortic syndrome.

[Metabolic syndrome and aortic stiffness].

Science.gov (United States)

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

2010-09-01

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

Outcome-based anatomic criteria for defining the hostile aortic neck.

Science.gov (United States)

Jordan, William D; Ouriel, Kenneth; Mehta, Manish; Varnagy, David; Moore, William M; Arko, Frank R; Joye, James; de Vries, Jean-Paul P M

2015-06-01

There is abundant evidence linking hostile proximal aortic neck anatomy to poor outcome after endovascular aortic aneurysm repair (EVAR), yet the definition of hostile anatomy varies from study to study. This current analysis was undertaken to identify anatomic criteria that are most predictive of success or failure at the aortic neck after EVAR. The study group comprised 221 patients in the Aneurysm Treatment using the Heli-FX Aortic Securement System Global Registry (ANCHOR) clinical trial, a population enriched with patients with challenging aortic neck anatomy and failure of sealing. Imaging protocols were not protocol specified but were performed according to the institution's standard of care. Core laboratory analysis assessed the three-dimensional centerline-reformatted computed tomography scans. Failure at the aortic neck was defined by type Ia endoleak occurring at the time of the initial endograft implantation or during follow-up. Receiver operating characteristic curve analysis was used to assess the value of each anatomic measure in the classification of aortic neck success and failure and to identify optimal thresholds of discrimination. Binary logistic regression was performed after excluding highly intercorrelated variables, creating a final model with significant predictors of outcome after EVAR. Among the 221 patients, 121 (54.8%) remained free of type Ia endoleak and 100 (45.2%) did not. Type Ia endoleaks presented immediately after endograft deployment in 58 (58.0%) or during follow-up in 42 (42.0%). Receiver operating characteristic curve analysis identified 12 variables where the classification of patients with type Ia endoleak was significantly more accurate than chance alone. Increased aortic neck diameter at the lowest renal artery (P = .013) and at 5 mm (P = .008), 10 mm (P = .008), and 15 mm (P = .010) distally; aneurysm sac diameter (P = .001), common iliac artery diameters (right, P = .012; left, P = .032), and a conical (P = .049) neck

CT diagnosis of acute aortic dissection

International Nuclear Information System (INIS)

Ogawa, Noriko; Kobayashi, Takeshi

1989-01-01

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

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

Science.gov (United States)

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

2011-08-01

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

Left main coronary artery obstruction by dislodged native-valve calculus after transcatheter aortic valve replacement.

Science.gov (United States)

Durmaz, Tahir; Ayhan, Huseyin; Keles, Telat; Aslan, Abdullah Nabi; Erdogan, Kemal Esref; Sari, Cenk; Bilen, Emine; Akcay, Murat; Bozkurt, Engin

2014-08-01

Transcatheter aortic valve replacement can be an effective, reliable treatment for severe aortic stenosis in surgically high-risk or ineligible patients. However, various sequelae like coronary artery obstruction can occur, not only in the long term, but also immediately after the procedure. We present the case of a 78-year-old woman whose left main coronary artery became obstructed with calculus 2 hours after the transfemoral implantation of an Edwards Sapien XT aortic valve. Despite percutaneous coronary intervention in that artery, the patient died. This case reminds us that early recognition of acute coronary obstruction and prompt intervention are crucial in patients with aortic stenosis who have undergone transcatheter aortic valve replacement.

[Exploration for micro-osteotomy assisted orthodontic treatment of skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region].

Science.gov (United States)

Wang, Bo; Shen, Guo-fang; Fang, Bing; Sun, Liang-yan; Wu, Yong; Jiang, Ling-yong; Zhu, Min

2012-10-01

To investigate the changes of periodontal conditions after micro-osteotomy assisted lower incisor decompensation for skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region. The sample consisted of 22 cases diagnosed as skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region, selected from consecutive patients of Department of Oral & Cranio-maxillofacial Science of Shanghai Ninth People's Hospital during 2009-2012. The samples were divided into 2 groups; G1 comprised 10 patients who accepted micro-osteotomy assisted lower incisor decompensation; G2 comprised 12 patients who chose traditional pre-surgical decomposition. The changes of periodontal conditions of both groups were evaluated with the help of cone-beam CT(CBCT). Data was processed using SAS8.02 software package. For subjects in G1, during the micro-osteotomy assisted pre-surgical orthodontics, no significant difference was found in the amount of root resorption of lower incisors.But labial and lingual vertical alveolar bone loss were 2.60 mm and 2.22 mm; alveolar bone thickness increased by 3.05 mm on the labial side and decreased by 0.88 mm on the lingual side (Ppre-surgical orthodontics was much safer than traditional orthodontics for skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region.

Aortic Graft Infection Secondary to Iatrogenic Transcolonic Graft Malposition.

Science.gov (United States)

Blank, Jacqueline J; Rothstein, Abby E; Lee, Cheong Jun; Malinowski, Michael J; Lewis, Brian D; Ridolfi, Timothy J; Otterson, Mary F

2018-01-01

Aortic graft infections are a rare but devastating complication of aortic revascularization. Often infections occur due to contamination at the time of surgery. Iatrogenic misplacement of the limbs of an aortobifemoral graft is exceedingly rare, and principles of evaluation and treatment are not well defined. We report 2 cases of aortobifemoral bypass graft malposition through the colon. Case 1 is a 54-year-old male who underwent aortobifemoral bypass grafting for acute limb ischemia. He had previously undergone a partial sigmoid colectomy for diverticulitis. Approximately 6 months after vascular surgery, he presented with an occult graft infection. Preoperative imaging and intraoperative findings were consistent with graft placement through the sigmoid colon. Case 2 is a 60-year-old male who underwent aortobifemoral bypass grafting due to a nonhealing wound after toe amputation. His postoperative course was complicated by pneumonia, bacteremia thought to be secondary to the pneumonia, general malaise, and persistent fevers. Approximately 10 weeks after the vascular surgery, he presented with imaging and intraoperative findings of graft malposition through the cecum. Aortic graft infection is usually caused by surgical contamination and presents as an indolent infection. Case 1 presented as such; Case 2 presented more acutely. Both grafts were iatrogenically misplaced through the colon at the index operation. The patients underwent extra-anatomic bypass and graft explantation and subsequently recovered.

Case report: ruptured aortic aneurysm into oesophagus - treatment by covered stent

International Nuclear Information System (INIS)

Young, N.; Tan, I.; Costa, R.

2002-01-01

Full text: A case report of a 70 year old man acutely presenting with a large descending thoracic aortic aneurysm, ruptured into the adjacent oesophagus. He was treated with an Aneurex covered stent (Medtronics). This patient has a long history of ischaemic heart disease, hypertension and previously treated abdominal aortic aneurysm. After diagnosis by CT, the bleeding was emergency temponaded by insertion of a Sengstaken-Blackmore tube into the oesophagus and inflated to arterial pressure. After stabilisation in ICU, it was decided that open surgery would probably prove fatal, and insertion of a covered thoracic aortic stent was performed in theatre with a femoral artery cut-down. Post-operatively the bleeding from the aortic aneurysm ceased. However, the patient has ongoing problems with large, open oesophageal ulcer with chronic ooze requiring intermittent transfusions, chronic infection with MRSA, requiring long term antibiotics, feeding via feeding gastrostomy tubes. The patient is stable 12 months after presentation. Treatment of this otherwise fatal problem by covered stent has permitted survival benefit. However, there are significant, unresolved issues of oesophageal ulcer and ongoing MRSA infection. Copyright (2002) Blackwell Science Pty Ltd

Midterm outcome of valve-sparing aortic root replacement in inherited connective tissue disorders.

Science.gov (United States)

Tanaka, Hiroshi; Ogino, Hitoshi; Matsuda, Hitoshi; Minatoya, Kenji; Sasaki, Hiroaki; Iba, Yutaka

2011-11-01

This study determined the midterm outcome of valve-sparing aortic root replacement for patients with inherited connective tissue disorders. From 1993 to 2008, 94 patients underwent valve-sparing aortic root replacement. Sixty patients (64%), average age 33 years (range, 15 to 61 years), had inherited connective tissue disorders: Marfan syndrome, 54 (92%); Loeys-Dietz syndrome, 5 (8%); and smooth muscle α-actin (ACTA2) mutation in 1. Median preoperative sinus diameter was 52 mm (range, 42 to 76 mm), and moderate/severe aortic regurgitation was present in 14 (23%). Seven (12%, 1993 to 1999) underwent remodeling procedures, and 53 had reimplantation procedures. Cusp repair was performed in 4. Median follow-up was 55 months (range, 1 to 149 months). There were 15 patients in the early term (1993 to 2000) and 45 in the late term (2001 to 2008). Four late deaths occurred (cardiac, 3; aortic, 1), with 10-year survival of 86%. Rates of freedom from aortic valve replacement at 5 and 10 years were 85% and 58% in remodeling and 96% and 58% in reimplantation. Risk factors for reoperations were postprocedure intraoperative aortic insufficiency greater than mild (p = 0.046), remodeling procedure (p = 0.016), and early term (p = 0.0002). One patient (2%) with none/trivial postprocedure aortic insufficiency required aortic valve replacement. Freedom from reoperation in patients with none/trivial postprocedure aortic insufficiency at 5 and 10 years was 100% and 67%. Meticulous control of aortic insufficiency during operation would bring favorable midterm durability in valve-sparing aortic root replacement using a reimplantation technique, even in patients with inherited connective tissue disorders. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

New mutations of DAX-1 genes in two Japanese patients with X-linked congenital adrenal hypoplasia and hypogonadotropic hypogonadism

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Yanase, Toshihiko; Takayanagi, Ryoichi; Oba, Koichi [Kyushu Univ., Fukuoka (Japan)] [and others

1996-02-01

Congenital adrenal hypoplasia, an X-linked disorder, is characterized by primary adrenal insufficiency and frequent association with hypogonadotropic hypogonadism. The X-chromosome gene DAX-1 has been most recently identified and shown to be responsible for this disorder. We analyzed the DAX-1 genes of two unrelated Japanese patients with congenital adrenal hypoplasia and hypogonadotropic hypogonadism by using PCR amplification of genomic DNA and its complete exonic sequencing. In a family containing several affected individuals, the proband male patient had a stop codon (TGA) in place of tryptophan (TGG) at amino acid position 171. As expected, his mother was a heterozygous carrier for the mutation, whereas his father and unaffected brother did not carry this mutation. In another male patient with noncontributory family history, sequencing revealed a 1-bp (T) deletion at amino acid position 280, leading to a frame shift and, subsequently a premature stop codon at amino acid position 371. The presence of this mutation in the patients` genome was further confirmed by digestion of genomic PCR product with MspI created by this mutation. Family studies using MspI digestion of genomic PCR products revealed that neither parent of this individual carried the mutation. These results clearly indicate that congenital adrenal hypoplasia and hypogonadotropic hypogonadism result from not only inherited but also de novo mutation in the DAX-1 gene. 31 refs., 4 figs., 2 tabs.

Development of stroke-induced quadriplegia after endovascular repair of blunt aortic injury pseudoaneurysm.

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Amoudi, Abdullah S; Merdad, Anas A; Makhdoom, Ahmed Q; Jamjoom, Reda A

2015-01-01

Endovascular repair of blunt aortic injury is now a first-line approach in management. This can warrant coverage of the left subclavian artery (LSA), which could lead to posterior strokes. In this case report, we present a severe complication of endovascular repair of a traumatic aortic aneurysm. A 53-year-old man presented with blunt aortic injury, endovascular repair was carried out where the left subclavian artery was covered. The intervention had a 100% technical success. Twelve hours later, he was discovered to have quadriplegia, a CT scan showed a large left cerebellar infarction extending to the medulla oblongata and proximal spinal cord. Strokes complicate 3% of thoracic endovascular aortic repairs, 80% of those strokes occur in patients who had their LSA`s covered. Most patients however, tolerate the coverage. Although our patient had a dominant right vertebral artery, and lacked risks for these strokes, he developed an extensive stroke that left him quadriplegic.

Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome.

Science.gov (United States)

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

2015-09-29

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

Over 20 years experience with aortic homograft in aortic valve replacement during acute infective endocarditis.

Science.gov (United States)

Solari, Silvia; Mastrobuoni, Stefano; De Kerchove, Laurent; Navarra, Emiliano; Astarci, Parla; Noirhomme, Philippe; Poncelet, Alain; Jashari, Ramadan; Rubay, Jean; El Khoury, Gebrine

2016-12-01

Despite the controversy, the aortic homograft is supposedly the best option in acute infective endocarditis (AIE), due to its resistance to reinfection. However, the technical complexity and the risk of structural deterioration over time have limited its utilization. The aim of this study was to evaluate the long-term results of aortic homograft for the treatment of infective endocarditis in our institution with particular attention to predictors of survival and homograft reoperation. The cohort includes 112 patients who underwent aortic valve replacement with an aortic homograft for AIE between January 1990 and December 2014. Fifteen patients (13.4%) died during the first 30 days after the operation. Two patients were lost to follow-up after discharge from the hospital; therefore, 95 patients were available for long-term analysis. The median duration of follow-up was 7.8 years (IQR 4.7-17.6). Five patients (5.3%) suffered a recurrence of infective endocarditis (1 relapse and 4 new episodes). Sixteen patients (16.8%) were reoperated for structural valve degeneration (SVD; n = 14, 87.5%) or for infection recurrence (n = 2, 12.5%). Freedom from homograft reoperation for infective endocarditis or structural homograft degeneration at 10 and 15 years postoperatively was 86.3 ± 5.5 and 47.3 ± 11.0%, respectively. For patients requiring homograft reoperation, the median interval to reintervention was 11.6 years (IQR 8.3-14.5). Long-term survival was 63.6% (95% CI 52.4-72.8%) and 53.8% (95% CI 40.6-65.3%) at 10 and 15 years, respectively. The use of aortic homograft in acute aortic valve endocarditis is associated with a remarkably low risk of relapsing infection and very acceptable long-term survival. The risk of reoperation due to SVD is significant after one decade especially in young patients. The aortic homograft seems to be ideally suited for reconstruction of the aortic valve and cardiac structures damaged by the infective process especially in early surgery. �

PREVALENCE AND PREDICTORS OF CORONARY ARTERY DISEASE IN SEVERE AORTIC STENOSIS PATIENTS UNDERGOING SURGERY

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Narayanapilai Jayaprasad

2017-09-01

Full Text Available BACKGROUND The present study is to find out the prevalence of CAD in severe Aortic Stenosis (AS patients undergoing aortic valve replacement and the relation between the conventional risk factors and CAD in them. Calcific aortic stenosis is associated with the same risk factors as coronary artery disease. Studies on calcific aortic stenosis and CAD are rarely reported from India. The aim of the study is to find out the predictive value of angina for presence of CAD in severe aortic stenosis. MATERIALS AND METHODS All cases of isolated severe aortic stenosis undergoing CAG before aortic valve replacement were included in the study. Data regarding various risk factors, symptoms, echocardiographic parameters and angiographic profile were collected. RESULTS Among a total of 94 patients who have undergone aortic valve replacement for severe AS 40 (42.6% had CAD. Risk factors like advanced age, hypertension, diabetes and smoking were more in patients with CAD compared to isolate AS. Mean gradient was more in the AS + CAD group compared with AS group. Presence of angina had a sensitivity of 82.5% and specificity of 51.85% for predicting coexisting CAD. CONCLUSION Among severe aortic stenosis, patients undergoing AVR 42.6% of patients had significant obstructive CAD. The conventional risk factors predict presence of coronary artery disease. Angina has got a fairly good sensitivity, but moderate specificity for CAD.

Tobacco smoking and aortic aneurysm

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

2013-01-01

BACKGROUND: We determined the predictive power of tobacco smoking on aortic aneurysm as opposed to other risk factors in the general population. METHODS: We recorded tobacco smoking and other risk factors at baseline, and assessed hospitalization and death from aortic aneurysm in 15,072 individuals...... aneurysm in males and females consuming above 20g tobacco daily was 3.5% and 1.3%, among those >60years with plasma cholesterol >5mmol/L and a systolic blood pressure >140mmHg. CONCLUSIONS: Tobacco smoking is the most important predictor of future aortic aneurysm outcomes in the general population...

Treatment strategy for ruptured abdominal aortic aneurysms.

Science.gov (United States)

Davidovic, L

2014-07-01

Rupture is the most serious and lethal complication of the abdominal aortic aneurysm. Despite all improvements during the past 50 years, ruptured abdominal aortic aneurysms are still associated with very high mortality. Namely, including patients who die before reaching the hospital, the mortality rate due to abdominal aortic aneurysm rupture is 90%. On the other hand, during the last twenty years, the number of abdominal aortic aneurysms significantly increased. One of the reasons is the fact that in majority of countries the general population is older nowadays. Due to this, the number of degenerative AAA is increasing. This is also the case for patients with abdominal aortic aneurysm rupture. Age must not be the reason of a treatment refusal. Optimal therapeutic option ought to be found. The following article is based on literature analysis including current guidelines but also on my Clinics significant experience. Furthermore, this article show cases options for vascular medicine in undeveloped countries that can not apply endovascular procedures at a sufficient level and to a sufficient extent. At this moment the following is evident. Thirty-day-mortality after repair of ruptured abdominal aortic aneurysms is significantly lower in high-volume hospitals. Due to different reasons all ruptured abdominal aortic aneurysms are not suitable for EVAR. Open repair of ruptured abdominal aortic aneurysm should be performed by experienced open vascular surgeons. This could also be said for the treatment of endovascular complications that require open surgical conversion. There is no ideal procedure for the treatment of AAA. Each has its own advantages and disadvantages, its own limits and complications, as well as indications and contraindications. Future reductions in mortality of ruptured abdominal aortic aneurysms will depend on implementation of population-based screening; on strategies to prevent postoperative organ injury and also on new medical technology

Effect of candesartan treatment on left ventricular remodeling after aortic valve replacement for aortic stenosis

DEFF Research Database (Denmark)

Dahl, Jordi S; Videbaek, Lars; Poulsen, Mikael K

2010-01-01

In hypertension, angiotensin receptor blockers can augment regression of left ventricular (LV) hypertrophy. It is not known whether this also is the case after aortic valve replacement (AVR) for severe aortic stenosis (AS). To test the hypothesis that treatment with candesartan in addition to con...

Statins for aortic valve stenosis

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Luciana Thiago

Full Text Available ABSTRACT BACKGROUND: Aortic valve stenosis is the most common type of valvular heart disease in the USA and Europe. Aortic valve stenosis is considered similar to atherosclerotic disease. Some studies have evaluated statins for aortic valve stenosis. OBJECTIVES: To evaluate the effectiveness and safety of statins in aortic valve stenosis. METHODS: Search methods: We searched the Cochrane Central Register of Controlled Trials (CENTRAL, MEDLINE, Embase, LILACS - IBECS, Web of Science and CINAHL Plus. These databases were searched from their inception to 24 November 2015. We also searched trials in registers for ongoing trials. We used no language restrictions. Selection criteria: Randomized controlled clinical trials (RCTs comparing statins alone or in association with other systemic drugs to reduce cholesterol levels versus placebo or usual care. Data collection and analysis: Primary outcomes were severity of aortic valve stenosis (evaluated by echocardiographic criteria: mean pressure gradient, valve area and aortic jet velocity, freedom from valve replacement and death from cardiovascular cause. Secondary outcomes were hospitalization for any reason, overall mortality, adverse events and patient quality of life. Two review authors independently selected trials for inclusion, extracted data and assessed the risk of bias. The GRADE methodology was employed to assess the quality of result findings and the GRADE profiler (GRADEPRO was used to import data from Review Manager 5.3 to create a 'Summary of findings' table. MAIN RESULTS: We included four RCTs with 2360 participants comparing statins (1185 participants with placebo (1175 participants. We found low-quality evidence for our primary outcome of severity of aortic valve stenosis, evaluated by mean pressure gradient (mean difference (MD -0.54, 95% confidence interval (CI -1.88 to 0.80; participants = 1935; studies = 2, valve area (MD -0.07, 95% CI -0.28 to 0.14; participants = 127; studies = 2

Statins for aortic valve stenosis.

Science.gov (United States)

Thiago, Luciana; Tsuji, Selma Rumiko; Nyong, Jonathan; Puga, Maria Eduarda Dos Santos; Góis, Aécio Flávio Teixeira de; Macedo, Cristiane Rufino; Valente, Orsine; Atallah, Álvaro Nagib

2016-01-01

Aortic valve stenosis is the most common type of valvular heart disease in the USA and Europe. Aortic valve stenosis is considered similar to atherosclerotic disease. Some studies have evaluated statins for aortic valve stenosis. To evaluate the effectiveness and safety of statins in aortic valve stenosis. Search methods: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, LILACS - IBECS, Web of Science and CINAHL Plus. These databases were searched from their inception to 24 November 2015. We also searched trials in registers for ongoing trials. We used no language restrictions.Selection criteria: Randomized controlled clinical trials (RCTs) comparing statins alone or in association with other systemic drugs to reduce cholesterol levels versus placebo or usual care. Data collection and analysis: Primary outcomes were severity of aortic valve stenosis (evaluated by echocardiographic criteria: mean pressure gradient, valve area and aortic jet velocity), freedom from valve replacement and death from cardiovascular cause. Secondary outcomes were hospitalization for any reason, overall mortality, adverse events and patient quality of life.Two review authors independently selected trials for inclusion, extracted data and assessed the risk of bias. The GRADE methodology was employed to assess the quality of result findings and the GRADE profiler (GRADEPRO) was used to import data from Review Manager 5.3 to create a 'Summary of findings' table. We included four RCTs with 2360 participants comparing statins (1185 participants) with placebo (1175 participants). We found low-quality evidence for our primary outcome of severity of aortic valve stenosis, evaluated by mean pressure gradient (mean difference (MD) -0.54, 95% confidence interval (CI) -1.88 to 0.80; participants = 1935; studies = 2), valve area (MD -0.07, 95% CI -0.28 to 0.14; participants = 127; studies = 2), and aortic jet velocity (MD -0.06, 95% CI -0.26 to 0

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

Science.gov (United States)

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

2005-08-24

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

The intraventricular filling vortex under heightened aortic blood pressure

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Nelsen, Nicholas; Gaddam, Manikantam; Santhanakrishnan, Arvind

2017-11-01

Hypertension, or high aortic blood pressure, can induce structural changes in the left ventricle (LV) such as concentric hypertrophy. Previous studies have identified that the intraventricular filling vortex serves as an effective means of blood transport during diastolic filling. However, a fundamental understanding of how hypertension affects this vortex is unavailable. This knowledge can be useful for improving diagnosis and treatment of related heart disease conditions, including hypertensive heart failure. In this experimental study, we hypothesized that the circulation of the filling vortex would diminish with increased aortic pressure. Using a LV physical model within a left heart simulator, we performed hemodynamic measurements to acquire pressure and volumetric inflow profiles and 2D particle image velocimetry to visualize the intraventricular flow fields. Peak aortic pressures of 120 mm Hg, 140 mm Hg, and 160 mm Hg were each tested at heart rates of 70, 100, and 110 beats per minute, under: 1) reduced ejection fraction (EF), and 2) constant EF. Our results indicate that peak vortex circulation is reduced under elevated aortic pressures. Hemodynamics and characteristics of the intraventricular filling vortex in all examined experimental cases will be presented.

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

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Ming-Hsun Yang

2009-05-01

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

Diagnosis of mycotic abdominal aortic aneurysm using 67-gallium citrate

International Nuclear Information System (INIS)

Blumoff, R.L.; McCartney, W.; Jaques, P.; Johnson, G. Jr.

1982-01-01

Mycotic aneurysms of the abdominal aorta are uncommon, but potentially lethal problems. Clinical subtleties may suggest their presence, but in the past, definitive diagnosis has been dependent on surgical exploration or autopsy findings. A case is presented in which 67-gallium citrate abdominal scanning localized the site of sepsis in an abdominal aortic aneurysm and allowed for prompt and successful surgical therapy. This noninvasive technique is recommended as a adjunct in the diagnosis of mycotic abdominal aortic aneurysms

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

International Nuclear Information System (INIS)

Ahn, Kyung-Sik; Yong, Hwan Seok; Woo, Ok Hee; Kang, Eun-Young; Lee, Joo-Won

2007-01-01

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

Arch translocation and the intra-arch elephant-trunk technique with collared graft for extended chronic dissecting aortic aneurysm

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Ikenaga Shigeru

2013-01-01

Full Text Available Abstract Management of extensive, chronic, dissecting aortic aneurysms after prior repair of the ascending aorta presents a technical challenge for surgeons. A symptomatic 64-year-old patient was admitted for elective surgical repair of an aortic annular dilatation, causing severe aortic regurgitation, and a Crawford type II extended thoracoabdominal aneurysm, 4 years after he underwent primary repair of an acute aortic dissection. The aorta was diffusely dilated, and there were no sites beyond the distal aortic arch where anastomosis could be performed. We successfully performed total aortic replacement with a 2-stage strategy, using an arch translocation technique and an intra-arch elephant-trunk technique.

Aortic embolization of an Edwards SAPIEN prosthesis due to sigmoid left ventricular hypertrophy: Case report.

Science.gov (United States)

Yuksel, Isa Öner; Koklu, Erkan; Arslan, Sakir; Cagirci, Goksel; Kucukseymen, Selcuk

2016-06-01

Transcatheter aortic valve implantation (TAVI) is considered an alternative therapy in high-risk patients with severe aortic stenosis. Although a minimally invasive procedure, it is not free from complications, one of which is valve embolization at the time of TAVI. We present a case of embolization of a balloon-expandable aortic valve due to sigmoid left ventricular hypertrophy and managed with a second valve without surgery. The embolized valve was repositioned in the aortic arch between the left common carotid artery and the brachiocephalic trunk. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

Congenital dislocation of the deep digital flexor tendon associated with hypoplasia of the sustentaculum tali in a Thoroughbred colt

International Nuclear Information System (INIS)

Lepage, O.M.; Leveille, R.; Breton, L.; Marcoux, M.

1995-01-01

An 11-month-old Thoroughbred colt was presented with a hard swelling at the proximal third of the right 4th metatarsal bone. A medial dislocation of the deep digital flexor tendon (flexor digitorum profundus) was also observed on the same leg. On the plantaroproximal-plantarodistal projection of the calcaneus, there was flattening and shortening of the sustentaculum tali. The smooth bony proliferation at the proximal third of the right 4th metatarus was compatible with a chronic splint bone fracture. This report describes a medial deep digital flexor dislocation associated with hypoplasia of the sustentaculum tali

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

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 AVC. The number of calcified coronary arteries correlated with ARC (rho = 0.45, p AVC. 29/152 patients had echocardiographic evidence of BAV and 123 TAV, who were older (p

Is there a prospect for hybrid aortic arch surgery?

Science.gov (United States)

Bashir, Mohamad; Harky, Amer; Bilal, Haris

2018-05-16

The surge of endovascular repair of aortic aneurysm in current modern aortic surgery practice has been the key for surgical management of elective cases of thoracic aortic aneurysms. This has paved way for the combined hybrid approach to be amongst the armamentarium for the management of aortic arch disease. The pivotal understanding of the aortic arch natural history coupled with device technology advancement allowed surgeons insight into delivery of hybrid surgery with acceptable morbidity and mortality results. This review article provides current insights into hybrid technique of aortic arch aneurysm repair and the evidences behind its applicability to arch surgery. It is aimed to highlight the challenges encountered for this innovative approach and correlate its challenges to those that are met by the conventional open aortic arch repair.

Hyperattenuating aortic wall on postmortem computed tomography (PMCT)

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Shiotani, Seiji; Kohno, Mototsugu; Ohashi, Noriyoshi; Yamazaki, Kentaroh; Nakayama, Hidetsugu; Ito, Yoshiyuki; Kaga, Kazunori; Ebashi, Toshio [Tsukuba Medical Center Hospital, Ibaraki (Japan); Itai, Yuji [Tsukuba Univ., Ibaraki (Japan). Inst. of Clinical Medicine

2002-08-01

The purpose of this study was to quantitatively evaluate the finding of hyperattenuating aortic wall on postmortem computed tomography (PMCT) and investigate its causes. Our subjects were 50 PMCT of non-traumatic deaths and 50 CT of living persons (live CT). The ascending aorta at the level of the carina was visually assessed regarding the presence or absence of hyperattenuating aortic wall and hematocrit effect on PMCT and live CT. The diameter, thickness of the aortic wall, and CT number (HU) of the aortic wall and the lumen were also measured. Hyperattenuating aortic wall was detected in 100% of PMCT and 2% of live CT. The diameter of the aortic wall was 2.9{+-}0.5 cm on PMCT and 3.5{+-}0.5 cm on live CT, showing a significant difference. The thickness of the aortic wall was 2 mm on PMCT. Hematocrit effect was observed in 46% of PMCT and in none of live CT. With PMCT, there was a significant difference between the CT numbers of the upper and lower half portions of the lumen (19.6{+-}11.7/30.9{+-}12.9), whereas, with live CT, there was no such significant difference (37.4{+-}7.6/38.9{+-}6.7), with the overall value of 38.2{+-}6.7. The CT number of the aortic wall was 49.9{+-}10.9 on PMCT. The causes of hyperattenuating aortic wall on PMCT are considered to be increased attenuation due to contraction of the aortic wall, a lack of motion artifact, and decreased attenuation of the lumen due to dilution of blood after massive infusion at the time of cardiopulmonary resuscitation. (author)

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

The cardiovascular effects of aortic clamping and unclamping

African Journals Online (AJOL)

aortic clamping is that afterload and blood pressure increase, and ... individually, albeit they interact with each other. The degree of ... position of the aortic clamp, the greater the increase ... the increase in preload in response to aortic clamping.

[Surgical results for aortic involvement in Marfan syndrome].